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Improving Veteran Adherence to Preadmission ERAS Protocol: Decreasing Avoidable Surgical Cancellations and Post-Operative Length of Stay (LOS)
Purpose
Improve veteran adherence of preadmission enhanced recovery after surgery (ERAS) protocol.
Background
NMVAHCS implemented the multidisciplinary Enhanced Recovery After Surgery (ERAS) protocol in 2018 to reduce postoperative morbidity and LOS utilizing evidence-based practice. Perioperative, intraoperative and postoperative practices were adopted, and well adhered. However, preadmission preparedness, the veteran’s responsibility, lacked adherence. Although detailed verbal and written instructions were provided, improvements were necessary. Patient related issues (PRI) regarding anticoagulation, drivers, anesthesia preop, COVID testing, and preparation often led to surgical cancellations.
Methods
ANNIE, an approved mobile application (app) utilizing Short Message Service (SMS) texts was identified to engage veterans. After facility and Office of Connected Care approval, an automated program was designed to text veteran’s preadmission instructions. Messages include 1-way reminders and 2-way messages providing automated instructions based on response. Veteran’s consent and enroll in the app 1 week prior to surgery and receive daily reminders for prehabilitation: daily exercise, arranging driver, and refraining from smoking, alcohol, illicit and herbal medications. Two-way messages verify anesthesia pre-op appointment and anticoagulation status. Reply messages provide information for scheduling or instructions regarding anticoagulation. Texts verify receipt and understanding of bowel preparation medications, COVID testing, “clears” diet, and assess for COVID symptoms. The day prior to admission, time sensitive reminders alert the veteran to each step of the Nichol’s preparation and carbohydrate drink consumption. Messages continue post-operatively assessing status, encouraging activity and pulmonary toilet. Messages also verify discharge education, receipt of stoma supplies, and surgical follow-up appointment.
Results
Prior to ERAS the average LOS was 11 days, which was reduced to 9 days after initial protocol implementation. Veterans enrolled in the app averaged a LOS of 7 days: a cost savings of $31,865.00 per veteran and increased bed availability for other veterans awaiting surgery. In FY19, 69 avoidable PRI led to surgical cancellations. Cancellations decrease access to care and maintain avoidable facility costs averaging $30,270.00 per case. ERAS and enrollment in ANNIE decreased cancellations by 62% (26 cases) in FY20 and 70% (21 cases) in FY21.
Conclusions
Engaging veterans with SMS messages improves preadmission ERAS adherence: improving outcomes for the veteran and facility.
Purpose
Improve veteran adherence of preadmission enhanced recovery after surgery (ERAS) protocol.
Background
NMVAHCS implemented the multidisciplinary Enhanced Recovery After Surgery (ERAS) protocol in 2018 to reduce postoperative morbidity and LOS utilizing evidence-based practice. Perioperative, intraoperative and postoperative practices were adopted, and well adhered. However, preadmission preparedness, the veteran’s responsibility, lacked adherence. Although detailed verbal and written instructions were provided, improvements were necessary. Patient related issues (PRI) regarding anticoagulation, drivers, anesthesia preop, COVID testing, and preparation often led to surgical cancellations.
Methods
ANNIE, an approved mobile application (app) utilizing Short Message Service (SMS) texts was identified to engage veterans. After facility and Office of Connected Care approval, an automated program was designed to text veteran’s preadmission instructions. Messages include 1-way reminders and 2-way messages providing automated instructions based on response. Veteran’s consent and enroll in the app 1 week prior to surgery and receive daily reminders for prehabilitation: daily exercise, arranging driver, and refraining from smoking, alcohol, illicit and herbal medications. Two-way messages verify anesthesia pre-op appointment and anticoagulation status. Reply messages provide information for scheduling or instructions regarding anticoagulation. Texts verify receipt and understanding of bowel preparation medications, COVID testing, “clears” diet, and assess for COVID symptoms. The day prior to admission, time sensitive reminders alert the veteran to each step of the Nichol’s preparation and carbohydrate drink consumption. Messages continue post-operatively assessing status, encouraging activity and pulmonary toilet. Messages also verify discharge education, receipt of stoma supplies, and surgical follow-up appointment.
Results
Prior to ERAS the average LOS was 11 days, which was reduced to 9 days after initial protocol implementation. Veterans enrolled in the app averaged a LOS of 7 days: a cost savings of $31,865.00 per veteran and increased bed availability for other veterans awaiting surgery. In FY19, 69 avoidable PRI led to surgical cancellations. Cancellations decrease access to care and maintain avoidable facility costs averaging $30,270.00 per case. ERAS and enrollment in ANNIE decreased cancellations by 62% (26 cases) in FY20 and 70% (21 cases) in FY21.
Conclusions
Engaging veterans with SMS messages improves preadmission ERAS adherence: improving outcomes for the veteran and facility.
Purpose
Improve veteran adherence of preadmission enhanced recovery after surgery (ERAS) protocol.
Background
NMVAHCS implemented the multidisciplinary Enhanced Recovery After Surgery (ERAS) protocol in 2018 to reduce postoperative morbidity and LOS utilizing evidence-based practice. Perioperative, intraoperative and postoperative practices were adopted, and well adhered. However, preadmission preparedness, the veteran’s responsibility, lacked adherence. Although detailed verbal and written instructions were provided, improvements were necessary. Patient related issues (PRI) regarding anticoagulation, drivers, anesthesia preop, COVID testing, and preparation often led to surgical cancellations.
Methods
ANNIE, an approved mobile application (app) utilizing Short Message Service (SMS) texts was identified to engage veterans. After facility and Office of Connected Care approval, an automated program was designed to text veteran’s preadmission instructions. Messages include 1-way reminders and 2-way messages providing automated instructions based on response. Veteran’s consent and enroll in the app 1 week prior to surgery and receive daily reminders for prehabilitation: daily exercise, arranging driver, and refraining from smoking, alcohol, illicit and herbal medications. Two-way messages verify anesthesia pre-op appointment and anticoagulation status. Reply messages provide information for scheduling or instructions regarding anticoagulation. Texts verify receipt and understanding of bowel preparation medications, COVID testing, “clears” diet, and assess for COVID symptoms. The day prior to admission, time sensitive reminders alert the veteran to each step of the Nichol’s preparation and carbohydrate drink consumption. Messages continue post-operatively assessing status, encouraging activity and pulmonary toilet. Messages also verify discharge education, receipt of stoma supplies, and surgical follow-up appointment.
Results
Prior to ERAS the average LOS was 11 days, which was reduced to 9 days after initial protocol implementation. Veterans enrolled in the app averaged a LOS of 7 days: a cost savings of $31,865.00 per veteran and increased bed availability for other veterans awaiting surgery. In FY19, 69 avoidable PRI led to surgical cancellations. Cancellations decrease access to care and maintain avoidable facility costs averaging $30,270.00 per case. ERAS and enrollment in ANNIE decreased cancellations by 62% (26 cases) in FY20 and 70% (21 cases) in FY21.
Conclusions
Engaging veterans with SMS messages improves preadmission ERAS adherence: improving outcomes for the veteran and facility.
Evaluating Progression Free Survival Among Veteran Population With Stage IV Non-Small Cell Immunotherapy vs Chemo- Immunotherapy
Background
Use of immune checkpoint inhibitors against advanced stage NSCLC has been associated with significant reduction in overall disease morbidity and mortality. However, despite the significant survival benefit, tumors invariably relapse. It is important to understand the pattern of progression and the progression free survival (PFS) to better predict disease outcomes and modify treatment approach.
Methods
We performed a retrospective review of 74 veterans with new diagnosis of stage IV NSCLC who received 2 or more cycles of immunotherapy with/without concurrent chemotherapy between 2015-2021 at the Stratton VA Medical Center. IRB approval was obtained. Fisher exact probability test and Kaplan-Meier estimators were used to analyze data with level of significance P < .05.
Results
Out of 74 patients, 38 patients were identified who received immunotherapy alone (Group A; n = 23, 60.5%) vs chemo-immunotherapy (Group B; n = 15, 39.5%). Baseline characteristics of Group A revealed median age 70 (IQR, 65-78), adenocarcinoma (n = 10, 43.4%), squamous cell carcinoma (n = 12, 52.1%), PD-L1 > 50% expression (n = 21, 91.3%), molecular testing positive for EGFR in 1 patient, otherwise negative for ROS, ALK, EGFR and BRAF mutations in all patients. Similarly, in Group B, median age 66 (IQR, 63-72), adenocarcinoma (n = 6, 40%), squamous cell carcinoma (n = 8, 53.3%), PD-L1 > 50% expression (n = 3, 20%), no mutations noted on molecular testing. Out of 38 patients, disease progression was noted in 19 patients, 10 in Group A (progression at initial site and new site n = 5, 50%) vs 9 in Group B (progression at initial site and new site, n = 6, 66.7%). Most common sites of progression included local and distant lymph nodes, brain, bone, and liver. Using the Kaplan-Meier analysis, median progression free survival (PFS) from start of immunotherapy till evidence of progression on imaging was 11 months in Group A and 7 months in Group B, P = .22. Our study recognized widespread metastases at the time of diagnosis (P = .03) as a possible factor affecting progression of diseases in Group A compared to Group B.
Conclusion
We conclude that although no statistically significant association was noted between the progression free survival between the two groups, the increased median PFS in immunotherapy only group is worth additional investigation. We recommend further large-scale studies to explore this association.
Background
Use of immune checkpoint inhibitors against advanced stage NSCLC has been associated with significant reduction in overall disease morbidity and mortality. However, despite the significant survival benefit, tumors invariably relapse. It is important to understand the pattern of progression and the progression free survival (PFS) to better predict disease outcomes and modify treatment approach.
Methods
We performed a retrospective review of 74 veterans with new diagnosis of stage IV NSCLC who received 2 or more cycles of immunotherapy with/without concurrent chemotherapy between 2015-2021 at the Stratton VA Medical Center. IRB approval was obtained. Fisher exact probability test and Kaplan-Meier estimators were used to analyze data with level of significance P < .05.
Results
Out of 74 patients, 38 patients were identified who received immunotherapy alone (Group A; n = 23, 60.5%) vs chemo-immunotherapy (Group B; n = 15, 39.5%). Baseline characteristics of Group A revealed median age 70 (IQR, 65-78), adenocarcinoma (n = 10, 43.4%), squamous cell carcinoma (n = 12, 52.1%), PD-L1 > 50% expression (n = 21, 91.3%), molecular testing positive for EGFR in 1 patient, otherwise negative for ROS, ALK, EGFR and BRAF mutations in all patients. Similarly, in Group B, median age 66 (IQR, 63-72), adenocarcinoma (n = 6, 40%), squamous cell carcinoma (n = 8, 53.3%), PD-L1 > 50% expression (n = 3, 20%), no mutations noted on molecular testing. Out of 38 patients, disease progression was noted in 19 patients, 10 in Group A (progression at initial site and new site n = 5, 50%) vs 9 in Group B (progression at initial site and new site, n = 6, 66.7%). Most common sites of progression included local and distant lymph nodes, brain, bone, and liver. Using the Kaplan-Meier analysis, median progression free survival (PFS) from start of immunotherapy till evidence of progression on imaging was 11 months in Group A and 7 months in Group B, P = .22. Our study recognized widespread metastases at the time of diagnosis (P = .03) as a possible factor affecting progression of diseases in Group A compared to Group B.
Conclusion
We conclude that although no statistically significant association was noted between the progression free survival between the two groups, the increased median PFS in immunotherapy only group is worth additional investigation. We recommend further large-scale studies to explore this association.
Background
Use of immune checkpoint inhibitors against advanced stage NSCLC has been associated with significant reduction in overall disease morbidity and mortality. However, despite the significant survival benefit, tumors invariably relapse. It is important to understand the pattern of progression and the progression free survival (PFS) to better predict disease outcomes and modify treatment approach.
Methods
We performed a retrospective review of 74 veterans with new diagnosis of stage IV NSCLC who received 2 or more cycles of immunotherapy with/without concurrent chemotherapy between 2015-2021 at the Stratton VA Medical Center. IRB approval was obtained. Fisher exact probability test and Kaplan-Meier estimators were used to analyze data with level of significance P < .05.
Results
Out of 74 patients, 38 patients were identified who received immunotherapy alone (Group A; n = 23, 60.5%) vs chemo-immunotherapy (Group B; n = 15, 39.5%). Baseline characteristics of Group A revealed median age 70 (IQR, 65-78), adenocarcinoma (n = 10, 43.4%), squamous cell carcinoma (n = 12, 52.1%), PD-L1 > 50% expression (n = 21, 91.3%), molecular testing positive for EGFR in 1 patient, otherwise negative for ROS, ALK, EGFR and BRAF mutations in all patients. Similarly, in Group B, median age 66 (IQR, 63-72), adenocarcinoma (n = 6, 40%), squamous cell carcinoma (n = 8, 53.3%), PD-L1 > 50% expression (n = 3, 20%), no mutations noted on molecular testing. Out of 38 patients, disease progression was noted in 19 patients, 10 in Group A (progression at initial site and new site n = 5, 50%) vs 9 in Group B (progression at initial site and new site, n = 6, 66.7%). Most common sites of progression included local and distant lymph nodes, brain, bone, and liver. Using the Kaplan-Meier analysis, median progression free survival (PFS) from start of immunotherapy till evidence of progression on imaging was 11 months in Group A and 7 months in Group B, P = .22. Our study recognized widespread metastases at the time of diagnosis (P = .03) as a possible factor affecting progression of diseases in Group A compared to Group B.
Conclusion
We conclude that although no statistically significant association was noted between the progression free survival between the two groups, the increased median PFS in immunotherapy only group is worth additional investigation. We recommend further large-scale studies to explore this association.
Commentary: Combination Chemotherapies, September 2022
In the PD-L1 CPS ≥ 1 subgroup, however, no significant OS benefit was observed (median OS 17.6 months vs 16.0 months; HR 0.86; 95% CI 0.72-1.04; P = .1125). Additionally, in an exploratory analysis, the addition of pembrolizumab showed consistent OS benefit among patients whose tumors express PD-L1 with a CPS of 10-19 and CPS ≥ 20. The updated progression-free survival (PFS) and objective response rates (ORR) were consistent with prior interim data. No new safety signals were observed after the longer follow-up.
These data confirm that pembrolizumab plus chemotherapy should remain the first-line treatment for patients with advanced or metastatic TNBC whose tumors express PD-L1 with a CPS of ≥ 10. The treatment of metastatic TNBC with low or negative PD-L1 CPS scores remains an area of unmet clinical need, and further research is needed to explore better options for these patients.
Wang and colleagues presented results from a randomized, phase 3 trial comparing first-line nab-paclitaxel plus cisplatin (AP) with gemcitabine plus cisplatin (GP) among 254 patients with previously untreated metastatic TNBC. Median PFS (mPFS) was 9.8 months with AP vs 7.4 months with GP (HR 0.67; 95% CI 0.50-0.88; P = .004). Furthermore, AP had significantly higher ORR compared with GP (81.1% vs 56.3%; P < .001) and significantly improved median OS (26.3 months vs 22.9 months; HR 0.62; 95% CI 0.44-0.90; P = .010).
In the exploratory analyses of PFS by stratification factors, the mPFS was significantly longer in the AP group compared with the GP group in the majority of subgroups, except for those patients who presented with de novo stage IV disease or a disease-free interval of < 1 year. Regarding safety data, a significantly higher incidence of grade ≥ 3 neuropathy (19% vs 0%) and nausea and vomiting (6% vs 1%) was noted in the AP group compared with the GP group, while grade ≥ 3 thrombocytopenia was more common in the GP group compared with the AP group (29.4% vs 3.9%).
The AP doublet achieved superior efficacy with a manageable safety profile, compared with GP in patients with previously untreated metastatic TNBC. It is not clear, however, whether the AP doublet is superior to single-agent therapy in this setting, especially given several prior studies that showed no survival benefit and increased toxicity from combination therapy compared with sequential single-agent therapy in metastatic breast cancer. More studies are needed to establish the role of the AP doublet in combination with pembrolizumab in this cohort of patients, given that first-line pembrolizumab plus chemotherapy is considered the standard of care for patients with metastatic TNBC whose tumors express PD-L1.
Rugo and colleagues presented results from a randomized phase 3 study comparing 205 mg/m2 oral paclitaxel plus 15 mg encequidar (a novel P-glycoprotein pump inhibitor that allows oral absorption of paclitaxel) on 3 consecutive days per week vs 175 mg/m2 intravenous paclitaxel once every 3 weeks. The study enrolled 402 postmenopausal women from Latin America with metastatic breast cancer who were at least 1 year from their last taxane therapy. Oral paclitaxel plus encequidar (oPac + E) increased the confirmed tumor response compared with intravenous paclitaxel (IVpac) (36% vs 23%; P = .01). There was a trend toward improved PFS (8.4 vs 7.4 months; HR 0.768; 95.5% CI 0.584-1.01; P = .046) and OS (22.7 vs 16.5 months; HR 0.794; 95.5% CI 0.607-1.037; P = .08) with oPac + E compared to IVpac, respectively. Grade ≥3 adverse events were comparable with oPac + E and IVpac (55% vs 53%), although a lower incidence of grade 3 neuropathy (2% vs 15%) and alopecia (49% vs 62%) was noted with oPac + E compared with IVpac. A higher incidence of grade ≥ 3 gastrointestinal toxicity (nausea, vomiting, and diarrhea) and grade 4 neutropenic complications was noted in the oPac + E group. Patients with elevated baseline liver enzymes were particularly susceptible to early neutropenia and serious infections.
This study demonstrates that oral paclitaxel can be a possible alternative treatment option to intravenous paclitaxel in a select group of patients with metastatic breast cancer. High-grade neutropenia appears to be a major treatment-limiting toxicity with oPac + E. Therefore, careful patient selection and close monitoring are crucial for the successful management of this adverse event.
In the PD-L1 CPS ≥ 1 subgroup, however, no significant OS benefit was observed (median OS 17.6 months vs 16.0 months; HR 0.86; 95% CI 0.72-1.04; P = .1125). Additionally, in an exploratory analysis, the addition of pembrolizumab showed consistent OS benefit among patients whose tumors express PD-L1 with a CPS of 10-19 and CPS ≥ 20. The updated progression-free survival (PFS) and objective response rates (ORR) were consistent with prior interim data. No new safety signals were observed after the longer follow-up.
These data confirm that pembrolizumab plus chemotherapy should remain the first-line treatment for patients with advanced or metastatic TNBC whose tumors express PD-L1 with a CPS of ≥ 10. The treatment of metastatic TNBC with low or negative PD-L1 CPS scores remains an area of unmet clinical need, and further research is needed to explore better options for these patients.
Wang and colleagues presented results from a randomized, phase 3 trial comparing first-line nab-paclitaxel plus cisplatin (AP) with gemcitabine plus cisplatin (GP) among 254 patients with previously untreated metastatic TNBC. Median PFS (mPFS) was 9.8 months with AP vs 7.4 months with GP (HR 0.67; 95% CI 0.50-0.88; P = .004). Furthermore, AP had significantly higher ORR compared with GP (81.1% vs 56.3%; P < .001) and significantly improved median OS (26.3 months vs 22.9 months; HR 0.62; 95% CI 0.44-0.90; P = .010).
In the exploratory analyses of PFS by stratification factors, the mPFS was significantly longer in the AP group compared with the GP group in the majority of subgroups, except for those patients who presented with de novo stage IV disease or a disease-free interval of < 1 year. Regarding safety data, a significantly higher incidence of grade ≥ 3 neuropathy (19% vs 0%) and nausea and vomiting (6% vs 1%) was noted in the AP group compared with the GP group, while grade ≥ 3 thrombocytopenia was more common in the GP group compared with the AP group (29.4% vs 3.9%).
The AP doublet achieved superior efficacy with a manageable safety profile, compared with GP in patients with previously untreated metastatic TNBC. It is not clear, however, whether the AP doublet is superior to single-agent therapy in this setting, especially given several prior studies that showed no survival benefit and increased toxicity from combination therapy compared with sequential single-agent therapy in metastatic breast cancer. More studies are needed to establish the role of the AP doublet in combination with pembrolizumab in this cohort of patients, given that first-line pembrolizumab plus chemotherapy is considered the standard of care for patients with metastatic TNBC whose tumors express PD-L1.
Rugo and colleagues presented results from a randomized phase 3 study comparing 205 mg/m2 oral paclitaxel plus 15 mg encequidar (a novel P-glycoprotein pump inhibitor that allows oral absorption of paclitaxel) on 3 consecutive days per week vs 175 mg/m2 intravenous paclitaxel once every 3 weeks. The study enrolled 402 postmenopausal women from Latin America with metastatic breast cancer who were at least 1 year from their last taxane therapy. Oral paclitaxel plus encequidar (oPac + E) increased the confirmed tumor response compared with intravenous paclitaxel (IVpac) (36% vs 23%; P = .01). There was a trend toward improved PFS (8.4 vs 7.4 months; HR 0.768; 95.5% CI 0.584-1.01; P = .046) and OS (22.7 vs 16.5 months; HR 0.794; 95.5% CI 0.607-1.037; P = .08) with oPac + E compared to IVpac, respectively. Grade ≥3 adverse events were comparable with oPac + E and IVpac (55% vs 53%), although a lower incidence of grade 3 neuropathy (2% vs 15%) and alopecia (49% vs 62%) was noted with oPac + E compared with IVpac. A higher incidence of grade ≥ 3 gastrointestinal toxicity (nausea, vomiting, and diarrhea) and grade 4 neutropenic complications was noted in the oPac + E group. Patients with elevated baseline liver enzymes were particularly susceptible to early neutropenia and serious infections.
This study demonstrates that oral paclitaxel can be a possible alternative treatment option to intravenous paclitaxel in a select group of patients with metastatic breast cancer. High-grade neutropenia appears to be a major treatment-limiting toxicity with oPac + E. Therefore, careful patient selection and close monitoring are crucial for the successful management of this adverse event.
In the PD-L1 CPS ≥ 1 subgroup, however, no significant OS benefit was observed (median OS 17.6 months vs 16.0 months; HR 0.86; 95% CI 0.72-1.04; P = .1125). Additionally, in an exploratory analysis, the addition of pembrolizumab showed consistent OS benefit among patients whose tumors express PD-L1 with a CPS of 10-19 and CPS ≥ 20. The updated progression-free survival (PFS) and objective response rates (ORR) were consistent with prior interim data. No new safety signals were observed after the longer follow-up.
These data confirm that pembrolizumab plus chemotherapy should remain the first-line treatment for patients with advanced or metastatic TNBC whose tumors express PD-L1 with a CPS of ≥ 10. The treatment of metastatic TNBC with low or negative PD-L1 CPS scores remains an area of unmet clinical need, and further research is needed to explore better options for these patients.
Wang and colleagues presented results from a randomized, phase 3 trial comparing first-line nab-paclitaxel plus cisplatin (AP) with gemcitabine plus cisplatin (GP) among 254 patients with previously untreated metastatic TNBC. Median PFS (mPFS) was 9.8 months with AP vs 7.4 months with GP (HR 0.67; 95% CI 0.50-0.88; P = .004). Furthermore, AP had significantly higher ORR compared with GP (81.1% vs 56.3%; P < .001) and significantly improved median OS (26.3 months vs 22.9 months; HR 0.62; 95% CI 0.44-0.90; P = .010).
In the exploratory analyses of PFS by stratification factors, the mPFS was significantly longer in the AP group compared with the GP group in the majority of subgroups, except for those patients who presented with de novo stage IV disease or a disease-free interval of < 1 year. Regarding safety data, a significantly higher incidence of grade ≥ 3 neuropathy (19% vs 0%) and nausea and vomiting (6% vs 1%) was noted in the AP group compared with the GP group, while grade ≥ 3 thrombocytopenia was more common in the GP group compared with the AP group (29.4% vs 3.9%).
The AP doublet achieved superior efficacy with a manageable safety profile, compared with GP in patients with previously untreated metastatic TNBC. It is not clear, however, whether the AP doublet is superior to single-agent therapy in this setting, especially given several prior studies that showed no survival benefit and increased toxicity from combination therapy compared with sequential single-agent therapy in metastatic breast cancer. More studies are needed to establish the role of the AP doublet in combination with pembrolizumab in this cohort of patients, given that first-line pembrolizumab plus chemotherapy is considered the standard of care for patients with metastatic TNBC whose tumors express PD-L1.
Rugo and colleagues presented results from a randomized phase 3 study comparing 205 mg/m2 oral paclitaxel plus 15 mg encequidar (a novel P-glycoprotein pump inhibitor that allows oral absorption of paclitaxel) on 3 consecutive days per week vs 175 mg/m2 intravenous paclitaxel once every 3 weeks. The study enrolled 402 postmenopausal women from Latin America with metastatic breast cancer who were at least 1 year from their last taxane therapy. Oral paclitaxel plus encequidar (oPac + E) increased the confirmed tumor response compared with intravenous paclitaxel (IVpac) (36% vs 23%; P = .01). There was a trend toward improved PFS (8.4 vs 7.4 months; HR 0.768; 95.5% CI 0.584-1.01; P = .046) and OS (22.7 vs 16.5 months; HR 0.794; 95.5% CI 0.607-1.037; P = .08) with oPac + E compared to IVpac, respectively. Grade ≥3 adverse events were comparable with oPac + E and IVpac (55% vs 53%), although a lower incidence of grade 3 neuropathy (2% vs 15%) and alopecia (49% vs 62%) was noted with oPac + E compared with IVpac. A higher incidence of grade ≥ 3 gastrointestinal toxicity (nausea, vomiting, and diarrhea) and grade 4 neutropenic complications was noted in the oPac + E group. Patients with elevated baseline liver enzymes were particularly susceptible to early neutropenia and serious infections.
This study demonstrates that oral paclitaxel can be a possible alternative treatment option to intravenous paclitaxel in a select group of patients with metastatic breast cancer. High-grade neutropenia appears to be a major treatment-limiting toxicity with oPac + E. Therefore, careful patient selection and close monitoring are crucial for the successful management of this adverse event.
Tips for navigating the altered retroperitoneum
Academic/Research Facility Utilization and Survival Outcomes in Osteosarcoma: An NCDB Analysis
Background
Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.
Methods
The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.
Results
Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).
Conclusions
This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.
Background
Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.
Methods
The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.
Results
Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).
Conclusions
This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.
Background
Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.
Methods
The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.
Results
Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).
Conclusions
This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.
Financial Toxicity in Colorectal Cancer Patient Who Received Localized Treatment in the Veterans Affairs Health System
Purpose
To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).
Background
CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.
Methods
We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.
Results
Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.
Conclusions/Implications
Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.
Purpose
To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).
Background
CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.
Methods
We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.
Results
Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.
Conclusions/Implications
Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.
Purpose
To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).
Background
CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.
Methods
We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.
Results
Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.
Conclusions/Implications
Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.
Impact of Insurance Status on Survival in Hurthle Cell Carcinoma: A National Cancer Database (NCDB) Analysis
Background
Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.
Methods
Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.
Results
We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.
Conclusions
This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.
Background
Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.
Methods
Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.
Results
We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.
Conclusions
This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.
Background
Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.
Methods
Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.
Results
We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.
Conclusions
This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.
Demographics in Early vs Late-Stage Laryngeal Squamous Cell Carcinoma: A NCDB Review
Purpose
To evaluate how various demographic factors impact the stage of cancer at diagnosis.
Background
Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.
Method/Analysis
Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.
Results
Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).
Conclusions/Implications
Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.
Purpose
To evaluate how various demographic factors impact the stage of cancer at diagnosis.
Background
Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.
Method/Analysis
Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.
Results
Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).
Conclusions/Implications
Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.
Purpose
To evaluate how various demographic factors impact the stage of cancer at diagnosis.
Background
Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.
Method/Analysis
Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.
Results
Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).
Conclusions/Implications
Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.
Not Just Castleman Disease: an Elusive Diagnosis of TAFRO Syndrome
Introduction
Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.
Case Report
A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.
Discussion
Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.
Introduction
Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.
Case Report
A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.
Discussion
Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.
Introduction
Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.
Case Report
A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.
Discussion
Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.
Waldenstrom Macroglobulinemia Presenting With Schnitzler Syndrome
Background
Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.
Case Report
A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.
Results
A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.
Background
Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.
Case Report
A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.
Results
A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.
Background
Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.
Case Report
A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.
Results
A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.