Hematology

Background

Department of Veterans Affairs (VA) faces a landscape of increasingly complex practice, especially in Hematology/Oncology (H/O), and a nationwide shortage of healthcare providers, while serving more Veterans than ever before. To understand current and future staffing needs, the VA National Oncology Program performed an assessment of H/O staffing, including attending physicians, residents/ fellows, licensed independent practitioners (LIPs) (nurse practitioners/physician assistants), and nurses for fiscal years (FY) 19–24.

Methods

Using VA Corporate Data Warehouse, we identified H/O visits in VA from 10/01/2018 through 09/30/2024 using stop codes. No-show (< 0.00001%) and National TeleOncology appointments (1%) were removed. We retrieved all notes associated with resulting visits and used area-ofspecialization and provider-type data to identify all attending physicians, trainees, LIPs, and nurses who authored or cosigned these notes. We identified H/O staff as 1. those associated with H/O clinic locations, 2. physicians who consistently cosigned H/O notes authored by fellows and LIPs associated with H/O locations, 3. fellows and LIPs authoring notes that were then cosigned by H/O physicians, and 4. nurses authoring notes associated with H/O visits.

Analysis

For each FY, we obtained total numbers of visits, unique patients, and care-providing staff by type. For validation, collaborating providers at several sites reviewed visit information, and a colleague also performed an independent, parallel data extraction. We adjusted FY totals to account for the growing patient population by dividing unique staff count by number of unique patients and multiplying by 200,000 (the approximate number of unique patients in FY19).

Results

From FY19 through FY24, VA Hematology/ Oncology saw a 14.6% rise in unique patients (from 232,084 to 265,926) and a 15.4% rise in visits (from 923,175 to 1,065,186). The absolute number of attendings rose by 4 (0.6%); of LIPs, by 138 (14.4%); and of nurses, by 142 (4.9%); trainees fell by 102 (4.3%). Adjusted to 200,000 patients, the number of attendings fell by 76 (12.3%); LIPs, by 1 (0.1%); trainees, by 335 (16.5%); and nurses, by 211 (8.4%).

Conclusions

Adjusted to number of Veterans, there are 10.4% fewer staff in Hematology/Oncology in FY24 compared to FY19.

Background

Department of Veterans Affairs (VA) faces a landscape of increasingly complex practice, especially in Hematology/Oncology (H/O), and a nationwide shortage of healthcare providers, while serving more Veterans than ever before. To understand current and future staffing needs, the VA National Oncology Program performed an assessment of H/O staffing, including attending physicians, residents/ fellows, licensed independent practitioners (LIPs) (nurse practitioners/physician assistants), and nurses for fiscal years (FY) 19–24.

Methods

Using VA Corporate Data Warehouse, we identified H/O visits in VA from 10/01/2018 through 09/30/2024 using stop codes. No-show (< 0.00001%) and National TeleOncology appointments (1%) were removed. We retrieved all notes associated with resulting visits and used area-ofspecialization and provider-type data to identify all attending physicians, trainees, LIPs, and nurses who authored or cosigned these notes. We identified H/O staff as 1. those associated with H/O clinic locations, 2. physicians who consistently cosigned H/O notes authored by fellows and LIPs associated with H/O locations, 3. fellows and LIPs authoring notes that were then cosigned by H/O physicians, and 4. nurses authoring notes associated with H/O visits.

Analysis

For each FY, we obtained total numbers of visits, unique patients, and care-providing staff by type. For validation, collaborating providers at several sites reviewed visit information, and a colleague also performed an independent, parallel data extraction. We adjusted FY totals to account for the growing patient population by dividing unique staff count by number of unique patients and multiplying by 200,000 (the approximate number of unique patients in FY19).

Results

From FY19 through FY24, VA Hematology/ Oncology saw a 14.6% rise in unique patients (from 232,084 to 265,926) and a 15.4% rise in visits (from 923,175 to 1,065,186). The absolute number of attendings rose by 4 (0.6%); of LIPs, by 138 (14.4%); and of nurses, by 142 (4.9%); trainees fell by 102 (4.3%). Adjusted to 200,000 patients, the number of attendings fell by 76 (12.3%); LIPs, by 1 (0.1%); trainees, by 335 (16.5%); and nurses, by 211 (8.4%).

Conclusions

Adjusted to number of Veterans, there are 10.4% fewer staff in Hematology/Oncology in FY24 compared to FY19.

Background

Department of Veterans Affairs (VA) faces a landscape of increasingly complex practice, especially in Hematology/Oncology (H/O), and a nationwide shortage of healthcare providers, while serving more Veterans than ever before. To understand current and future staffing needs, the VA National Oncology Program performed an assessment of H/O staffing, including attending physicians, residents/ fellows, licensed independent practitioners (LIPs) (nurse practitioners/physician assistants), and nurses for fiscal years (FY) 19–24.

Methods

Using VA Corporate Data Warehouse, we identified H/O visits in VA from 10/01/2018 through 09/30/2024 using stop codes. No-show (< 0.00001%) and National TeleOncology appointments (1%) were removed. We retrieved all notes associated with resulting visits and used area-ofspecialization and provider-type data to identify all attending physicians, trainees, LIPs, and nurses who authored or cosigned these notes. We identified H/O staff as 1. those associated with H/O clinic locations, 2. physicians who consistently cosigned H/O notes authored by fellows and LIPs associated with H/O locations, 3. fellows and LIPs authoring notes that were then cosigned by H/O physicians, and 4. nurses authoring notes associated with H/O visits.

Analysis

For each FY, we obtained total numbers of visits, unique patients, and care-providing staff by type. For validation, collaborating providers at several sites reviewed visit information, and a colleague also performed an independent, parallel data extraction. We adjusted FY totals to account for the growing patient population by dividing unique staff count by number of unique patients and multiplying by 200,000 (the approximate number of unique patients in FY19).

Results

From FY19 through FY24, VA Hematology/ Oncology saw a 14.6% rise in unique patients (from 232,084 to 265,926) and a 15.4% rise in visits (from 923,175 to 1,065,186). The absolute number of attendings rose by 4 (0.6%); of LIPs, by 138 (14.4%); and of nurses, by 142 (4.9%); trainees fell by 102 (4.3%). Adjusted to 200,000 patients, the number of attendings fell by 76 (12.3%); LIPs, by 1 (0.1%); trainees, by 335 (16.5%); and nurses, by 211 (8.4%).

Conclusions

Adjusted to number of Veterans, there are 10.4% fewer staff in Hematology/Oncology in FY24 compared to FY19.

Background

Accurate clinical coding that reflects all diagnoses and problems addressed during a patient encounter is essential for the cancer program’s data quality, research initiatives, and securing VERA (Veterans Equitable Resource Allocation) funding. However, providers often face barriers such as limited time during patient visits and difficulty navigating Electronic health record (EHR) systems. These challenges lead to inaccurate coding, which undermines downstream data integrity. This quality improvement (QI) study aimed to identify these barriers and implement an intervention to improve coding accuracy, while also assessing the financial implications of improved documentation.

Methods

This QI study was conducted at the Albany Stratton VA Medical Center, focusing on hematology/ oncology outpatient encounters. A baseline chart audit of diagnosis codes from June 2023 revealed an accuracy rate of 69.8%. To address this, an intervention was implemented in which dedicated coders were assigned to support attending physicians in coding for over a two-week period. These coders reviewed and corrected diagnosis codes in real-time. A follow-up audit conducted after the intervention showed an improved coding accuracy of 82%.

Discussion/Implications

Coding remains a timeconsuming task for providers, made more difficult by EHR systems that are not user-friendly. This study demonstrated that involving dedicated coders significantly improves documentation accuracy—from 69% to 82%. In addition to data quality, the financial benefits are notable. A projected annual return on investment of $216,094 was calculated, based on an internal analysis showing that in a sample of 124 patients, 10% could have qualified for higher VERA funding based on accurate coding, generating an estimated $17,427 in additional reimbursement per patient. This cost-benefit ratio supports the recommendation to staff dedicated coders. Other interventions were also utilised, such as updating the national encounter form and auto-populating documentation in Dragon software, but had limited impact and did not directly address diagnosis accuracy respectively.

Conclusions

Targeted interventions improved coding accuracy, but sustainability remains a challenge due to time and system limitations. Future efforts should focus on hiring full-time coders. These steps can further enhance coding quality and potentially increase hospital revenue.

Background

Accurate clinical coding that reflects all diagnoses and problems addressed during a patient encounter is essential for the cancer program’s data quality, research initiatives, and securing VERA (Veterans Equitable Resource Allocation) funding. However, providers often face barriers such as limited time during patient visits and difficulty navigating Electronic health record (EHR) systems. These challenges lead to inaccurate coding, which undermines downstream data integrity. This quality improvement (QI) study aimed to identify these barriers and implement an intervention to improve coding accuracy, while also assessing the financial implications of improved documentation.

Methods

This QI study was conducted at the Albany Stratton VA Medical Center, focusing on hematology/ oncology outpatient encounters. A baseline chart audit of diagnosis codes from June 2023 revealed an accuracy rate of 69.8%. To address this, an intervention was implemented in which dedicated coders were assigned to support attending physicians in coding for over a two-week period. These coders reviewed and corrected diagnosis codes in real-time. A follow-up audit conducted after the intervention showed an improved coding accuracy of 82%.

Discussion/Implications

Coding remains a timeconsuming task for providers, made more difficult by EHR systems that are not user-friendly. This study demonstrated that involving dedicated coders significantly improves documentation accuracy—from 69% to 82%. In addition to data quality, the financial benefits are notable. A projected annual return on investment of $216,094 was calculated, based on an internal analysis showing that in a sample of 124 patients, 10% could have qualified for higher VERA funding based on accurate coding, generating an estimated $17,427 in additional reimbursement per patient. This cost-benefit ratio supports the recommendation to staff dedicated coders. Other interventions were also utilised, such as updating the national encounter form and auto-populating documentation in Dragon software, but had limited impact and did not directly address diagnosis accuracy respectively.

Conclusions

Targeted interventions improved coding accuracy, but sustainability remains a challenge due to time and system limitations. Future efforts should focus on hiring full-time coders. These steps can further enhance coding quality and potentially increase hospital revenue.

Background

Accurate clinical coding that reflects all diagnoses and problems addressed during a patient encounter is essential for the cancer program’s data quality, research initiatives, and securing VERA (Veterans Equitable Resource Allocation) funding. However, providers often face barriers such as limited time during patient visits and difficulty navigating Electronic health record (EHR) systems. These challenges lead to inaccurate coding, which undermines downstream data integrity. This quality improvement (QI) study aimed to identify these barriers and implement an intervention to improve coding accuracy, while also assessing the financial implications of improved documentation.

Methods

This QI study was conducted at the Albany Stratton VA Medical Center, focusing on hematology/ oncology outpatient encounters. A baseline chart audit of diagnosis codes from June 2023 revealed an accuracy rate of 69.8%. To address this, an intervention was implemented in which dedicated coders were assigned to support attending physicians in coding for over a two-week period. These coders reviewed and corrected diagnosis codes in real-time. A follow-up audit conducted after the intervention showed an improved coding accuracy of 82%.

Discussion/Implications

Coding remains a timeconsuming task for providers, made more difficult by EHR systems that are not user-friendly. This study demonstrated that involving dedicated coders significantly improves documentation accuracy—from 69% to 82%. In addition to data quality, the financial benefits are notable. A projected annual return on investment of $216,094 was calculated, based on an internal analysis showing that in a sample of 124 patients, 10% could have qualified for higher VERA funding based on accurate coding, generating an estimated $17,427 in additional reimbursement per patient. This cost-benefit ratio supports the recommendation to staff dedicated coders. Other interventions were also utilised, such as updating the national encounter form and auto-populating documentation in Dragon software, but had limited impact and did not directly address diagnosis accuracy respectively.

Conclusions

Targeted interventions improved coding accuracy, but sustainability remains a challenge due to time and system limitations. Future efforts should focus on hiring full-time coders. These steps can further enhance coding quality and potentially increase hospital revenue.

Background

Due to risk for infusion-related reactions (IRR), administration of iron dextran requires an initial test dose with an extended monitoring period and subsequent doses given as a slow infusion over 2-3 hours. Safe use of a 60-minute iron dextran infusion protocol has been demonstrated previously at fully staffed academic teaching institutions. This study sought to determine the impact on patient safety and infusion clinic efficiency after implementing a 60-minute iron dextran administration protocol at a small, rural facility utilizing a decentralized clinical model.

Methods

This single-site, prospective, interventional study was conducted at a rural level 1C Veterans Affairs secondary care facility. The Hematology/Oncology clinic staffing includes one onsite clinical pharmacy practitioner (CPP) and advanced practice nurse. Remote providers complete patient encounters through video and telehealth modalities. A 60-minute iron dextran infusion service line agreement was designed by the Hematology/Oncology CPP and approved by the facility prior to data collection. The protocol included administration of a test dose and 15-minute monitoring period for treatment naïve patients. Pre-medications were allowed at the discretion of the ordering providers. All patients who received iron dextran between May 31, 2024 and April 14, 2025 per protocol were included in data analysis and results were stratified by treatment naïve and pre-treated patients. Outcomes included the proportion of patients experiencing any grade of IRR based on the Common Criteria for Adverse Events Version 5.0, and the average duration of administration. Descriptive statistics were used for safety and efficiency outcomes.

Results

Eighty patients received 103 iron dextran infusions and were included for analysis. Pre-medications were administered for 16 of the 103 (15.5%) included infusions. Two patients experienced grade 1 IRR (nausea) on 4 occasions (3.8%) which quickly resolved with intravenous ondansetron, and full iron dextran doses were received. The mean infusion time was 94 minutes in the treatment naïve cohort vs 71 minutes in the pre-treated cohort.

Conclusions

This study suggests a Hematology/ Oncology CPP developed iron dextran 60-minute infusion protocol may be safely and efficiently administered for qualifying patients in a decentralized, rural healthcare setting.

Background

Due to risk for infusion-related reactions (IRR), administration of iron dextran requires an initial test dose with an extended monitoring period and subsequent doses given as a slow infusion over 2-3 hours. Safe use of a 60-minute iron dextran infusion protocol has been demonstrated previously at fully staffed academic teaching institutions. This study sought to determine the impact on patient safety and infusion clinic efficiency after implementing a 60-minute iron dextran administration protocol at a small, rural facility utilizing a decentralized clinical model.

Methods

This single-site, prospective, interventional study was conducted at a rural level 1C Veterans Affairs secondary care facility. The Hematology/Oncology clinic staffing includes one onsite clinical pharmacy practitioner (CPP) and advanced practice nurse. Remote providers complete patient encounters through video and telehealth modalities. A 60-minute iron dextran infusion service line agreement was designed by the Hematology/Oncology CPP and approved by the facility prior to data collection. The protocol included administration of a test dose and 15-minute monitoring period for treatment naïve patients. Pre-medications were allowed at the discretion of the ordering providers. All patients who received iron dextran between May 31, 2024 and April 14, 2025 per protocol were included in data analysis and results were stratified by treatment naïve and pre-treated patients. Outcomes included the proportion of patients experiencing any grade of IRR based on the Common Criteria for Adverse Events Version 5.0, and the average duration of administration. Descriptive statistics were used for safety and efficiency outcomes.

Results

Eighty patients received 103 iron dextran infusions and were included for analysis. Pre-medications were administered for 16 of the 103 (15.5%) included infusions. Two patients experienced grade 1 IRR (nausea) on 4 occasions (3.8%) which quickly resolved with intravenous ondansetron, and full iron dextran doses were received. The mean infusion time was 94 minutes in the treatment naïve cohort vs 71 minutes in the pre-treated cohort.

Conclusions

This study suggests a Hematology/ Oncology CPP developed iron dextran 60-minute infusion protocol may be safely and efficiently administered for qualifying patients in a decentralized, rural healthcare setting.

Background

Due to risk for infusion-related reactions (IRR), administration of iron dextran requires an initial test dose with an extended monitoring period and subsequent doses given as a slow infusion over 2-3 hours. Safe use of a 60-minute iron dextran infusion protocol has been demonstrated previously at fully staffed academic teaching institutions. This study sought to determine the impact on patient safety and infusion clinic efficiency after implementing a 60-minute iron dextran administration protocol at a small, rural facility utilizing a decentralized clinical model.

Methods

This single-site, prospective, interventional study was conducted at a rural level 1C Veterans Affairs secondary care facility. The Hematology/Oncology clinic staffing includes one onsite clinical pharmacy practitioner (CPP) and advanced practice nurse. Remote providers complete patient encounters through video and telehealth modalities. A 60-minute iron dextran infusion service line agreement was designed by the Hematology/Oncology CPP and approved by the facility prior to data collection. The protocol included administration of a test dose and 15-minute monitoring period for treatment naïve patients. Pre-medications were allowed at the discretion of the ordering providers. All patients who received iron dextran between May 31, 2024 and April 14, 2025 per protocol were included in data analysis and results were stratified by treatment naïve and pre-treated patients. Outcomes included the proportion of patients experiencing any grade of IRR based on the Common Criteria for Adverse Events Version 5.0, and the average duration of administration. Descriptive statistics were used for safety and efficiency outcomes.

Results

Eighty patients received 103 iron dextran infusions and were included for analysis. Pre-medications were administered for 16 of the 103 (15.5%) included infusions. Two patients experienced grade 1 IRR (nausea) on 4 occasions (3.8%) which quickly resolved with intravenous ondansetron, and full iron dextran doses were received. The mean infusion time was 94 minutes in the treatment naïve cohort vs 71 minutes in the pre-treated cohort.

Conclusions

This study suggests a Hematology/ Oncology CPP developed iron dextran 60-minute infusion protocol may be safely and efficiently administered for qualifying patients in a decentralized, rural healthcare setting.

Purpose/Background

Palliative care referrals are recommended for patients with advanced or metastatic cancer to enhance patient and caregiver outcomes. However, challenges like delays or lack of referrals hinder implementation. This study identified rate of palliative care referrals at James A. Haley Veterans’ Hospital in Tampa, Florida; explored potential barriers to referral, and implemented targeted interventions to improve referral rates and patient outcomes.

Methods

A Plan-Do-Study-Act (PDSA) cycle was used for this quality improvement project. Data was collected from electronic medical record, focusing on consult dates, patient demographics, and reasons for seeking palliative care. Pre-intervention surveys were administered to Hematology-Oncology fellows at the institution to identify barriers to referral. Following a root cause analysis, a targeted intervention was developed, focusing on educational programs for fellows for streamlined referral processes.

Results

Before the intervention, monthly average for palliative care consults was low (3-8, typically 5). Pre-intervention surveys revealed that fellows lacked knowledge about palliative care resources, which contributed to low referral rates. To address this issue, a didactic session led by a palliative care specialist was conducted for the fellows in the fellowship program. This session provided education on the role of palliative care, how to initiate referrals, and the benefits of early involvement of palliative care teams in oncology patient management. Post-intervention surveys showed a marked improvement in fellows’ confidence regarding identification of patients suitable for palliative care. Following the session, 90% (9/10) of fellows reported being “very likely” to consult palliative care more often and 80% (8/10) indicated they were “very likely” to initiate palliative care discussions earlier in patient’s disease trajectory, with the remaining 20% (2/10) reporting a neutral stance. All fellows (100%) agreed that earlier palliative care involvement improves patient outcomes.

Implications/Significance

This PDSA cycle demonstrated that targeted education for fellows can increase awareness of palliative care resources and improve referral rates. Future work will focus on reassessing usage of palliative care consults post-intervention to evaluate effects of fellows’ education of appropriate palliative care consultation, make necessary interventions based on data and further evaluate the long-term impact on patient outcomes at James A. Haley Veterans’ Hospital.

Purpose/Background

Palliative care referrals are recommended for patients with advanced or metastatic cancer to enhance patient and caregiver outcomes. However, challenges like delays or lack of referrals hinder implementation. This study identified rate of palliative care referrals at James A. Haley Veterans’ Hospital in Tampa, Florida; explored potential barriers to referral, and implemented targeted interventions to improve referral rates and patient outcomes.

Methods

A Plan-Do-Study-Act (PDSA) cycle was used for this quality improvement project. Data was collected from electronic medical record, focusing on consult dates, patient demographics, and reasons for seeking palliative care. Pre-intervention surveys were administered to Hematology-Oncology fellows at the institution to identify barriers to referral. Following a root cause analysis, a targeted intervention was developed, focusing on educational programs for fellows for streamlined referral processes.

Results

Before the intervention, monthly average for palliative care consults was low (3-8, typically 5). Pre-intervention surveys revealed that fellows lacked knowledge about palliative care resources, which contributed to low referral rates. To address this issue, a didactic session led by a palliative care specialist was conducted for the fellows in the fellowship program. This session provided education on the role of palliative care, how to initiate referrals, and the benefits of early involvement of palliative care teams in oncology patient management. Post-intervention surveys showed a marked improvement in fellows’ confidence regarding identification of patients suitable for palliative care. Following the session, 90% (9/10) of fellows reported being “very likely” to consult palliative care more often and 80% (8/10) indicated they were “very likely” to initiate palliative care discussions earlier in patient’s disease trajectory, with the remaining 20% (2/10) reporting a neutral stance. All fellows (100%) agreed that earlier palliative care involvement improves patient outcomes.

Implications/Significance

This PDSA cycle demonstrated that targeted education for fellows can increase awareness of palliative care resources and improve referral rates. Future work will focus on reassessing usage of palliative care consults post-intervention to evaluate effects of fellows’ education of appropriate palliative care consultation, make necessary interventions based on data and further evaluate the long-term impact on patient outcomes at James A. Haley Veterans’ Hospital.

Purpose/Background

Palliative care referrals are recommended for patients with advanced or metastatic cancer to enhance patient and caregiver outcomes. However, challenges like delays or lack of referrals hinder implementation. This study identified rate of palliative care referrals at James A. Haley Veterans’ Hospital in Tampa, Florida; explored potential barriers to referral, and implemented targeted interventions to improve referral rates and patient outcomes.

Methods

A Plan-Do-Study-Act (PDSA) cycle was used for this quality improvement project. Data was collected from electronic medical record, focusing on consult dates, patient demographics, and reasons for seeking palliative care. Pre-intervention surveys were administered to Hematology-Oncology fellows at the institution to identify barriers to referral. Following a root cause analysis, a targeted intervention was developed, focusing on educational programs for fellows for streamlined referral processes.

Results

Before the intervention, monthly average for palliative care consults was low (3-8, typically 5). Pre-intervention surveys revealed that fellows lacked knowledge about palliative care resources, which contributed to low referral rates. To address this issue, a didactic session led by a palliative care specialist was conducted for the fellows in the fellowship program. This session provided education on the role of palliative care, how to initiate referrals, and the benefits of early involvement of palliative care teams in oncology patient management. Post-intervention surveys showed a marked improvement in fellows’ confidence regarding identification of patients suitable for palliative care. Following the session, 90% (9/10) of fellows reported being “very likely” to consult palliative care more often and 80% (8/10) indicated they were “very likely” to initiate palliative care discussions earlier in patient’s disease trajectory, with the remaining 20% (2/10) reporting a neutral stance. All fellows (100%) agreed that earlier palliative care involvement improves patient outcomes.

Implications/Significance

This PDSA cycle demonstrated that targeted education for fellows can increase awareness of palliative care resources and improve referral rates. Future work will focus on reassessing usage of palliative care consults post-intervention to evaluate effects of fellows’ education of appropriate palliative care consultation, make necessary interventions based on data and further evaluate the long-term impact on patient outcomes at James A. Haley Veterans’ Hospital.

Purpose/Background

The purpose of this project was to understand how implementing a consult template could optimize hematology E-consult evaluation. At the Tampa VA, providers can submit hematology E-consults for interpretation of lab abnormalities and management recommendations that do not require an in-person hematology evaluation. Previously, submission of an E-consult did not require prerequisite labs or imaging or for lab parameters to be met, leading to an increased number of hematology E-consults and subsequently, lower efficiency for hematologists.

Methods

A hematology E-consult template was created through collaboration between the hematology/ oncology and ambulatory care sections, which lists specific diagnoses and required parameters/workup needed for each diagnosis prior to submission of the E-consult. If those criteria were not met, the consult was cancelled. A representative sample of one month pre- and post-implementation data was analyzed.

Results

The E-consult template was implemented in September 2024. From April to August 2024, the average number of E-consults per month was 243, averaging at 11.0 per day, while from October 2024 to February 2025, the average number of E-consults per month was 146.4, averaging at 6.6 per day. In August 2024, the leading reasons for consult were anemia (77), leukocytosis (26), and thrombocytopenia (24). That month, there were 15 consult cancellations, with the primary reason being the patient was established in clinic (9). In October 2024, the leading reasons for consult were anemia (39), leukocytosis (14), and thrombocytopenia (13). That month, there were 34 consult cancellations, with the primary reason being that hematology advised a clinic consultation rather than an E-consult (10).

Implications/Significance

These data reveal that the hematology E-consult template was associated with a decreased number of E-consults per day and per month. Implementation of the hematology E-consult template allows the hematology consultants to focus on interpretation of lab results and providing management recommendations, as opposed to providing standard of care diagnostic recommendations. It also serves as an educational tool to referring providers, to understand appropriate indications for hematology E-consultation. Lastly, the template has created increased efficiency in providing hematology recommendations and ultimately, improved timely care for our veterans.

Purpose/Background

The purpose of this project was to understand how implementing a consult template could optimize hematology E-consult evaluation. At the Tampa VA, providers can submit hematology E-consults for interpretation of lab abnormalities and management recommendations that do not require an in-person hematology evaluation. Previously, submission of an E-consult did not require prerequisite labs or imaging or for lab parameters to be met, leading to an increased number of hematology E-consults and subsequently, lower efficiency for hematologists.

Methods

A hematology E-consult template was created through collaboration between the hematology/ oncology and ambulatory care sections, which lists specific diagnoses and required parameters/workup needed for each diagnosis prior to submission of the E-consult. If those criteria were not met, the consult was cancelled. A representative sample of one month pre- and post-implementation data was analyzed.

Results

The E-consult template was implemented in September 2024. From April to August 2024, the average number of E-consults per month was 243, averaging at 11.0 per day, while from October 2024 to February 2025, the average number of E-consults per month was 146.4, averaging at 6.6 per day. In August 2024, the leading reasons for consult were anemia (77), leukocytosis (26), and thrombocytopenia (24). That month, there were 15 consult cancellations, with the primary reason being the patient was established in clinic (9). In October 2024, the leading reasons for consult were anemia (39), leukocytosis (14), and thrombocytopenia (13). That month, there were 34 consult cancellations, with the primary reason being that hematology advised a clinic consultation rather than an E-consult (10).

Implications/Significance

These data reveal that the hematology E-consult template was associated with a decreased number of E-consults per day and per month. Implementation of the hematology E-consult template allows the hematology consultants to focus on interpretation of lab results and providing management recommendations, as opposed to providing standard of care diagnostic recommendations. It also serves as an educational tool to referring providers, to understand appropriate indications for hematology E-consultation. Lastly, the template has created increased efficiency in providing hematology recommendations and ultimately, improved timely care for our veterans.

Purpose/Background

The purpose of this project was to understand how implementing a consult template could optimize hematology E-consult evaluation. At the Tampa VA, providers can submit hematology E-consults for interpretation of lab abnormalities and management recommendations that do not require an in-person hematology evaluation. Previously, submission of an E-consult did not require prerequisite labs or imaging or for lab parameters to be met, leading to an increased number of hematology E-consults and subsequently, lower efficiency for hematologists.

Methods

A hematology E-consult template was created through collaboration between the hematology/ oncology and ambulatory care sections, which lists specific diagnoses and required parameters/workup needed for each diagnosis prior to submission of the E-consult. If those criteria were not met, the consult was cancelled. A representative sample of one month pre- and post-implementation data was analyzed.

Results

The E-consult template was implemented in September 2024. From April to August 2024, the average number of E-consults per month was 243, averaging at 11.0 per day, while from October 2024 to February 2025, the average number of E-consults per month was 146.4, averaging at 6.6 per day. In August 2024, the leading reasons for consult were anemia (77), leukocytosis (26), and thrombocytopenia (24). That month, there were 15 consult cancellations, with the primary reason being the patient was established in clinic (9). In October 2024, the leading reasons for consult were anemia (39), leukocytosis (14), and thrombocytopenia (13). That month, there were 34 consult cancellations, with the primary reason being that hematology advised a clinic consultation rather than an E-consult (10).

Implications/Significance

These data reveal that the hematology E-consult template was associated with a decreased number of E-consults per day and per month. Implementation of the hematology E-consult template allows the hematology consultants to focus on interpretation of lab results and providing management recommendations, as opposed to providing standard of care diagnostic recommendations. It also serves as an educational tool to referring providers, to understand appropriate indications for hematology E-consultation. Lastly, the template has created increased efficiency in providing hematology recommendations and ultimately, improved timely care for our veterans.

Case Presentation

A 75-year-old man with chronic kidney disease, hypertension and diabetes mellitus presented with acute kidney injury (creatinine 5.2 from baseline 4.2) and a two-week history of increased urinary frequency. Labs revealed high anion gap metabolic acidosis, proteinuria, hematuria, pyuria, and acute on chronic anemia. He was diagnosed with kappa light chain nephropathy and multiple myeloma with 32% plasma cells on bone marrow biopsy. He began treatment with bortezomib, cyclophosphamide, and dexamethasone (Cy- BorD). Three days after cyclophosphamide and five days after bortezomib, the patient developed persistent hypotension with systolic BP in the 50s, unresponsive to fluids and Trendelenburg position. Due to end-stage renal disease with anuria, fluid resuscitation was limited. He required norepinephrine and was transferred to the ICU. Given instability, hemodialysis was deferred, and continuous renal replacement therapy was initiated. Shock evaluation included a CT abdomen showing enteritis versus ileus; however, infectious workup was negative. Cardiogenic shock was ruled out with a serial echocardiogram showing normal ejection fractions of 59-67% without significant valvular disease. The workup for adrenal insufficiency was negative. After the exclusion of other potential causes of shock, severe refractory hypotension was attributed to bortezomib toxicity.Hypotension is a known adverse effect of bortezomib. Orthostatic hypotension may occur in 8 to 9% of patients, and rarely, patients may experience heart failure, conduction disorders and arrhythmias, or cardiogenic shock. The pathologic mechanism of this toxicity is still poorly understood. Proposed mechanisms include direct endothelial toxicity as evidenced by thrombotic microangiopathy or impairment of sympathetic and parasympathetic nerve fibres. Most commonly, patients experience neurotoxicity, which may manifest as autonomic dysfunction or peripheral neuropathy. Cardiovascular complications are typically reversible. Our patient’s cardiac function remained within normal limits; therefore, his persistent hypotension was felt to be the result of direct toxicity from bortezomib rather than cardiogenic shock. Ultimately, blood pressure did improve, and vasopressors were discontinued. However, he continued to have orthostatic hypotension and continued to require supportive fludrocortisone, midodrine, and pyridostigmine. Goals of care have been discussed, and he wished to continue pursuing restorative care, with a plan for transition to carfilzomib versus daratumumab outpatient.

Case Presentation

A 75-year-old man with chronic kidney disease, hypertension and diabetes mellitus presented with acute kidney injury (creatinine 5.2 from baseline 4.2) and a two-week history of increased urinary frequency. Labs revealed high anion gap metabolic acidosis, proteinuria, hematuria, pyuria, and acute on chronic anemia. He was diagnosed with kappa light chain nephropathy and multiple myeloma with 32% plasma cells on bone marrow biopsy. He began treatment with bortezomib, cyclophosphamide, and dexamethasone (Cy- BorD). Three days after cyclophosphamide and five days after bortezomib, the patient developed persistent hypotension with systolic BP in the 50s, unresponsive to fluids and Trendelenburg position. Due to end-stage renal disease with anuria, fluid resuscitation was limited. He required norepinephrine and was transferred to the ICU. Given instability, hemodialysis was deferred, and continuous renal replacement therapy was initiated. Shock evaluation included a CT abdomen showing enteritis versus ileus; however, infectious workup was negative. Cardiogenic shock was ruled out with a serial echocardiogram showing normal ejection fractions of 59-67% without significant valvular disease. The workup for adrenal insufficiency was negative. After the exclusion of other potential causes of shock, severe refractory hypotension was attributed to bortezomib toxicity.Hypotension is a known adverse effect of bortezomib. Orthostatic hypotension may occur in 8 to 9% of patients, and rarely, patients may experience heart failure, conduction disorders and arrhythmias, or cardiogenic shock. The pathologic mechanism of this toxicity is still poorly understood. Proposed mechanisms include direct endothelial toxicity as evidenced by thrombotic microangiopathy or impairment of sympathetic and parasympathetic nerve fibres. Most commonly, patients experience neurotoxicity, which may manifest as autonomic dysfunction or peripheral neuropathy. Cardiovascular complications are typically reversible. Our patient’s cardiac function remained within normal limits; therefore, his persistent hypotension was felt to be the result of direct toxicity from bortezomib rather than cardiogenic shock. Ultimately, blood pressure did improve, and vasopressors were discontinued. However, he continued to have orthostatic hypotension and continued to require supportive fludrocortisone, midodrine, and pyridostigmine. Goals of care have been discussed, and he wished to continue pursuing restorative care, with a plan for transition to carfilzomib versus daratumumab outpatient.

Case Presentation

A 75-year-old man with chronic kidney disease, hypertension and diabetes mellitus presented with acute kidney injury (creatinine 5.2 from baseline 4.2) and a two-week history of increased urinary frequency. Labs revealed high anion gap metabolic acidosis, proteinuria, hematuria, pyuria, and acute on chronic anemia. He was diagnosed with kappa light chain nephropathy and multiple myeloma with 32% plasma cells on bone marrow biopsy. He began treatment with bortezomib, cyclophosphamide, and dexamethasone (Cy- BorD). Three days after cyclophosphamide and five days after bortezomib, the patient developed persistent hypotension with systolic BP in the 50s, unresponsive to fluids and Trendelenburg position. Due to end-stage renal disease with anuria, fluid resuscitation was limited. He required norepinephrine and was transferred to the ICU. Given instability, hemodialysis was deferred, and continuous renal replacement therapy was initiated. Shock evaluation included a CT abdomen showing enteritis versus ileus; however, infectious workup was negative. Cardiogenic shock was ruled out with a serial echocardiogram showing normal ejection fractions of 59-67% without significant valvular disease. The workup for adrenal insufficiency was negative. After the exclusion of other potential causes of shock, severe refractory hypotension was attributed to bortezomib toxicity.Hypotension is a known adverse effect of bortezomib. Orthostatic hypotension may occur in 8 to 9% of patients, and rarely, patients may experience heart failure, conduction disorders and arrhythmias, or cardiogenic shock. The pathologic mechanism of this toxicity is still poorly understood. Proposed mechanisms include direct endothelial toxicity as evidenced by thrombotic microangiopathy or impairment of sympathetic and parasympathetic nerve fibres. Most commonly, patients experience neurotoxicity, which may manifest as autonomic dysfunction or peripheral neuropathy. Cardiovascular complications are typically reversible. Our patient’s cardiac function remained within normal limits; therefore, his persistent hypotension was felt to be the result of direct toxicity from bortezomib rather than cardiogenic shock. Ultimately, blood pressure did improve, and vasopressors were discontinued. However, he continued to have orthostatic hypotension and continued to require supportive fludrocortisone, midodrine, and pyridostigmine. Goals of care have been discussed, and he wished to continue pursuing restorative care, with a plan for transition to carfilzomib versus daratumumab outpatient.

Background

Marginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma most commonly arising in mucosal, nodal, or splenic tissues. While extranodal presentations are recognized, involvement of the sciatic foramen is exceedingly rare. We present a unique case of stage IV MZL with primary involvement of the left sciatic foramen, identified incidentally during urologic evaluation.

Case Presentation

A 74-year-old male patient was referred for hematologic evaluation after imaging revealed a left sciatic foraminal mass during work-up for elevated PSA. CT abdomen/pelvis revealed a 4.7 cm mass in the left sciatic foramen. Follow-up PET-CT confirmed hypermetabolic activity in the mass, with additional areas of uptake in the right ilium and pleural- pericardial regions. The patient was asymptomatic and denied B-symptoms. CT-guided biopsy of the sciatic mass revealed low-grade B-cell lymphoma. Flow cytometry showed a CD20-positive, CD5-negative, CD10-negative, lambda light chain–restricted population consistent with marginal zone lymphoma. Laboratory studies demonstrated iron deficiency anemia, with otherwise unremarkable counts and chemistries. He was started on monotherapy with rituximab for four cycles. He tolerated treatment well. Interval PET imaging in April 2025 showed stable disease in the sciatic foramen and mild improvement in pleural- pericardial uptake. He is planned to start obinutuzumab in the upcoming month.

Discussion

This case illustrates a rare anatomic presentation of MZL, likely representing primary sciatic foramen involvement. The presence of additional PETavid lesions complicates staging, raising consideration of stage I vs. III/IV disease. Biopsy was limited to the sciatic lesion, and no bone marrow sampling was performed. Given the patient’s excellent performance status, absence of symptoms, and low tumor burden, single-agent rituximab was chosen initially in accordance with NCCN guidelines.

Conclusions

Sciatic foramen involvement by MZL is an extremely rare occurrence and may mimic more common soft tissue or neurogenic tumors radiographically. This case underscores the importance of biopsy for diagnosis and the value of multidisciplinary care. In the veteran population, such incidental findings on imaging warrant comprehensive evaluation, particularly in atypical anatomical sites.

Background

Marginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma most commonly arising in mucosal, nodal, or splenic tissues. While extranodal presentations are recognized, involvement of the sciatic foramen is exceedingly rare. We present a unique case of stage IV MZL with primary involvement of the left sciatic foramen, identified incidentally during urologic evaluation.

Case Presentation

A 74-year-old male patient was referred for hematologic evaluation after imaging revealed a left sciatic foraminal mass during work-up for elevated PSA. CT abdomen/pelvis revealed a 4.7 cm mass in the left sciatic foramen. Follow-up PET-CT confirmed hypermetabolic activity in the mass, with additional areas of uptake in the right ilium and pleural- pericardial regions. The patient was asymptomatic and denied B-symptoms. CT-guided biopsy of the sciatic mass revealed low-grade B-cell lymphoma. Flow cytometry showed a CD20-positive, CD5-negative, CD10-negative, lambda light chain–restricted population consistent with marginal zone lymphoma. Laboratory studies demonstrated iron deficiency anemia, with otherwise unremarkable counts and chemistries. He was started on monotherapy with rituximab for four cycles. He tolerated treatment well. Interval PET imaging in April 2025 showed stable disease in the sciatic foramen and mild improvement in pleural- pericardial uptake. He is planned to start obinutuzumab in the upcoming month.

Discussion

This case illustrates a rare anatomic presentation of MZL, likely representing primary sciatic foramen involvement. The presence of additional PETavid lesions complicates staging, raising consideration of stage I vs. III/IV disease. Biopsy was limited to the sciatic lesion, and no bone marrow sampling was performed. Given the patient’s excellent performance status, absence of symptoms, and low tumor burden, single-agent rituximab was chosen initially in accordance with NCCN guidelines.

Conclusions

Sciatic foramen involvement by MZL is an extremely rare occurrence and may mimic more common soft tissue or neurogenic tumors radiographically. This case underscores the importance of biopsy for diagnosis and the value of multidisciplinary care. In the veteran population, such incidental findings on imaging warrant comprehensive evaluation, particularly in atypical anatomical sites.

Background

Marginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma most commonly arising in mucosal, nodal, or splenic tissues. While extranodal presentations are recognized, involvement of the sciatic foramen is exceedingly rare. We present a unique case of stage IV MZL with primary involvement of the left sciatic foramen, identified incidentally during urologic evaluation.

Case Presentation

A 74-year-old male patient was referred for hematologic evaluation after imaging revealed a left sciatic foraminal mass during work-up for elevated PSA. CT abdomen/pelvis revealed a 4.7 cm mass in the left sciatic foramen. Follow-up PET-CT confirmed hypermetabolic activity in the mass, with additional areas of uptake in the right ilium and pleural- pericardial regions. The patient was asymptomatic and denied B-symptoms. CT-guided biopsy of the sciatic mass revealed low-grade B-cell lymphoma. Flow cytometry showed a CD20-positive, CD5-negative, CD10-negative, lambda light chain–restricted population consistent with marginal zone lymphoma. Laboratory studies demonstrated iron deficiency anemia, with otherwise unremarkable counts and chemistries. He was started on monotherapy with rituximab for four cycles. He tolerated treatment well. Interval PET imaging in April 2025 showed stable disease in the sciatic foramen and mild improvement in pleural- pericardial uptake. He is planned to start obinutuzumab in the upcoming month.

Discussion

This case illustrates a rare anatomic presentation of MZL, likely representing primary sciatic foramen involvement. The presence of additional PETavid lesions complicates staging, raising consideration of stage I vs. III/IV disease. Biopsy was limited to the sciatic lesion, and no bone marrow sampling was performed. Given the patient’s excellent performance status, absence of symptoms, and low tumor burden, single-agent rituximab was chosen initially in accordance with NCCN guidelines.

Conclusions

Sciatic foramen involvement by MZL is an extremely rare occurrence and may mimic more common soft tissue or neurogenic tumors radiographically. This case underscores the importance of biopsy for diagnosis and the value of multidisciplinary care. In the veteran population, such incidental findings on imaging warrant comprehensive evaluation, particularly in atypical anatomical sites.

Background

Syncope presents a common diagnostic challenge due to its broad differential, ranging from benign to life-threatening conditions. Despite guidelines emphasizing a history- and physical examination- driven approach, nearly $33 billion is spent annually on syncope evaluations, often without yielding conclusive diagnoses. Here, we present a case of syncope secondary to cerebral venous sinus thrombosis, underscoring the importance of cerebrovascular imaging in select high-risk patient populations.

Case Presentation

An 80-year-old male with chronic sinusitis and history of pulmonary embolism (managed with thrombolysis and apixaban) presented due to an episode of transient loss of consciousness followed by nausea and vomiting. He denied any preceding symptoms but his wife did notice his arm move up for a few seconds. He didn’t have any headaches or post-ictal state. Physical exam showed normal orthostatic vital signs, symmetric blood pressure and radial pulses bilaterally. An extensive neurological exam was done and was unremarkable for any focal deficits including no vision changes. Initial evaluation including electrocardiogram, telemetry monitoring, transthoracic echocardiogram, and electroencephalography showed no significant abnormalities.

Chest CT angiography revealed right-sided segmental pulmonary emboli, unchanged from prior imaging. Head CT did not show any acute intracranial findings, and CT angiography demonstrated no vascular abnormality. Ultimately, an MRI brain revealed a left sigmoid sinus filling defect, suggestive of cerebral venous sinus thrombosis (CVST), in addition to chronic sinusitis. As CVST occurred while on apixaban, anticoagulation was switched to enoxaparin. He did not experience any recurrent symptoms during admission.

Conclusions

This case highlights the need for a patient- specific approach to syncope evaluation. Early neurovascular imaging may aid in prompt diagnosis and prevent unnecessary testing. CVST is a rare manifestation of venous thromboembolism and may present with symptoms mimicking vasovagal syncope, such as nausea and transient loss of consciousness. Typical symptoms of CVST include headaches, vomiting, vision changes, focal deficits, seizures, mental status changes, stupor or coma. Risk factors include prior thrombosis, hypercoagulable states like pregnancy or malignancy, obesity, OCPs, and chronic sinusitis. Noncontrast CT may miss CVST, and advanced neuroimaging is necessary for diagnosis in high-risk patients with thrombotic risk factors or symptoms suggestive of elevated intracranial pressure.

Background

Syncope presents a common diagnostic challenge due to its broad differential, ranging from benign to life-threatening conditions. Despite guidelines emphasizing a history- and physical examination- driven approach, nearly $33 billion is spent annually on syncope evaluations, often without yielding conclusive diagnoses. Here, we present a case of syncope secondary to cerebral venous sinus thrombosis, underscoring the importance of cerebrovascular imaging in select high-risk patient populations.

Case Presentation

An 80-year-old male with chronic sinusitis and history of pulmonary embolism (managed with thrombolysis and apixaban) presented due to an episode of transient loss of consciousness followed by nausea and vomiting. He denied any preceding symptoms but his wife did notice his arm move up for a few seconds. He didn’t have any headaches or post-ictal state. Physical exam showed normal orthostatic vital signs, symmetric blood pressure and radial pulses bilaterally. An extensive neurological exam was done and was unremarkable for any focal deficits including no vision changes. Initial evaluation including electrocardiogram, telemetry monitoring, transthoracic echocardiogram, and electroencephalography showed no significant abnormalities.

Chest CT angiography revealed right-sided segmental pulmonary emboli, unchanged from prior imaging. Head CT did not show any acute intracranial findings, and CT angiography demonstrated no vascular abnormality. Ultimately, an MRI brain revealed a left sigmoid sinus filling defect, suggestive of cerebral venous sinus thrombosis (CVST), in addition to chronic sinusitis. As CVST occurred while on apixaban, anticoagulation was switched to enoxaparin. He did not experience any recurrent symptoms during admission.

Conclusions

This case highlights the need for a patient- specific approach to syncope evaluation. Early neurovascular imaging may aid in prompt diagnosis and prevent unnecessary testing. CVST is a rare manifestation of venous thromboembolism and may present with symptoms mimicking vasovagal syncope, such as nausea and transient loss of consciousness. Typical symptoms of CVST include headaches, vomiting, vision changes, focal deficits, seizures, mental status changes, stupor or coma. Risk factors include prior thrombosis, hypercoagulable states like pregnancy or malignancy, obesity, OCPs, and chronic sinusitis. Noncontrast CT may miss CVST, and advanced neuroimaging is necessary for diagnosis in high-risk patients with thrombotic risk factors or symptoms suggestive of elevated intracranial pressure.

Background

Syncope presents a common diagnostic challenge due to its broad differential, ranging from benign to life-threatening conditions. Despite guidelines emphasizing a history- and physical examination- driven approach, nearly $33 billion is spent annually on syncope evaluations, often without yielding conclusive diagnoses. Here, we present a case of syncope secondary to cerebral venous sinus thrombosis, underscoring the importance of cerebrovascular imaging in select high-risk patient populations.

Case Presentation

An 80-year-old male with chronic sinusitis and history of pulmonary embolism (managed with thrombolysis and apixaban) presented due to an episode of transient loss of consciousness followed by nausea and vomiting. He denied any preceding symptoms but his wife did notice his arm move up for a few seconds. He didn’t have any headaches or post-ictal state. Physical exam showed normal orthostatic vital signs, symmetric blood pressure and radial pulses bilaterally. An extensive neurological exam was done and was unremarkable for any focal deficits including no vision changes. Initial evaluation including electrocardiogram, telemetry monitoring, transthoracic echocardiogram, and electroencephalography showed no significant abnormalities.

Chest CT angiography revealed right-sided segmental pulmonary emboli, unchanged from prior imaging. Head CT did not show any acute intracranial findings, and CT angiography demonstrated no vascular abnormality. Ultimately, an MRI brain revealed a left sigmoid sinus filling defect, suggestive of cerebral venous sinus thrombosis (CVST), in addition to chronic sinusitis. As CVST occurred while on apixaban, anticoagulation was switched to enoxaparin. He did not experience any recurrent symptoms during admission.

Conclusions

This case highlights the need for a patient- specific approach to syncope evaluation. Early neurovascular imaging may aid in prompt diagnosis and prevent unnecessary testing. CVST is a rare manifestation of venous thromboembolism and may present with symptoms mimicking vasovagal syncope, such as nausea and transient loss of consciousness. Typical symptoms of CVST include headaches, vomiting, vision changes, focal deficits, seizures, mental status changes, stupor or coma. Risk factors include prior thrombosis, hypercoagulable states like pregnancy or malignancy, obesity, OCPs, and chronic sinusitis. Noncontrast CT may miss CVST, and advanced neuroimaging is necessary for diagnosis in high-risk patients with thrombotic risk factors or symptoms suggestive of elevated intracranial pressure.

Background

Whereas light chain (AL) amyloid is the most common cause of amyloidosis, Apolipoprotein A-IV (AApoAIV) amyloid is rare. Here we describe the first case of renal amyloidosis with pathology demonstrating concurrent deposition of AL and AApoAIV amyloid.

Case Presentation

A 79-year-old man presented with bilateral leg swelling and was diagnosed with nephrotic syndrome with preserved renal function. Serum protein electrophoresis and immunofixation showed no measurable monoclonal protein. Serum lambda free light chains were elevated at 97.72 mg/L, and kappa:lambda ratio was 0.22. Pathology from renal biopsy showed focal deposits of Congo red staining. Liquid chromatography tandem mass spectrometry identified both fibrillogenic ApoIV signal sequence peptides and a peptide profile consistent with AL amyloid deposition.

Further testing showed mild Bence Jones proteinuria, mildly elevated beta-2 microglobulin, elevated cardiac troponin T, normal NT-pro-B-type natriuretic peptide, and normal Factor X activity. Echocardiogram and FDG PET CT were unremarkable. Bone marrow biopsy demonstrated a lambda-restricted monotypic plasma cell population comprising 10% of plasma cells by immunohistochemistry. Although the Congo red stain was indeterminate, IGH::CCND1 fusion was detected by FISH.

Treatment was initiated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone. Serum lambda light chains normalized after one cycle. Urine protein:creatinine ratio improved from 7.0 to 3.0 by the end of cycle 3. The patient will soon complete cycle 4 and undergo reassessment.

Discussion

While there are rare reports of AApoAIV renal amyloidosis and more prevalent cases of AL renal amyloidosis, the presence of both types of amyloid in the same patient has not been previously reported. AApoAIV renal amyloidosis has no established treatment and tends to present with rising serum creatinine as opposed to proteinuria. Given our patient’s clinical presentation with nephrotic syndrome and current light chain response to treatment, AL amyloidosis may be predominantly driving the disease.

Conclusions

Given the rarity of renal amyloidosis involving both AL and AApoAIV amyloid, the clinical course and optimal treatments are not established. The cumulative knowledge gained from individual case studies can serve as a basis for ongoing investigation and management of similar cases in the future.

Background

Whereas light chain (AL) amyloid is the most common cause of amyloidosis, Apolipoprotein A-IV (AApoAIV) amyloid is rare. Here we describe the first case of renal amyloidosis with pathology demonstrating concurrent deposition of AL and AApoAIV amyloid.

Case Presentation

A 79-year-old man presented with bilateral leg swelling and was diagnosed with nephrotic syndrome with preserved renal function. Serum protein electrophoresis and immunofixation showed no measurable monoclonal protein. Serum lambda free light chains were elevated at 97.72 mg/L, and kappa:lambda ratio was 0.22. Pathology from renal biopsy showed focal deposits of Congo red staining. Liquid chromatography tandem mass spectrometry identified both fibrillogenic ApoIV signal sequence peptides and a peptide profile consistent with AL amyloid deposition.

Further testing showed mild Bence Jones proteinuria, mildly elevated beta-2 microglobulin, elevated cardiac troponin T, normal NT-pro-B-type natriuretic peptide, and normal Factor X activity. Echocardiogram and FDG PET CT were unremarkable. Bone marrow biopsy demonstrated a lambda-restricted monotypic plasma cell population comprising 10% of plasma cells by immunohistochemistry. Although the Congo red stain was indeterminate, IGH::CCND1 fusion was detected by FISH.

Treatment was initiated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone. Serum lambda light chains normalized after one cycle. Urine protein:creatinine ratio improved from 7.0 to 3.0 by the end of cycle 3. The patient will soon complete cycle 4 and undergo reassessment.

Discussion

While there are rare reports of AApoAIV renal amyloidosis and more prevalent cases of AL renal amyloidosis, the presence of both types of amyloid in the same patient has not been previously reported. AApoAIV renal amyloidosis has no established treatment and tends to present with rising serum creatinine as opposed to proteinuria. Given our patient’s clinical presentation with nephrotic syndrome and current light chain response to treatment, AL amyloidosis may be predominantly driving the disease.

Conclusions

Given the rarity of renal amyloidosis involving both AL and AApoAIV amyloid, the clinical course and optimal treatments are not established. The cumulative knowledge gained from individual case studies can serve as a basis for ongoing investigation and management of similar cases in the future.

Background

Whereas light chain (AL) amyloid is the most common cause of amyloidosis, Apolipoprotein A-IV (AApoAIV) amyloid is rare. Here we describe the first case of renal amyloidosis with pathology demonstrating concurrent deposition of AL and AApoAIV amyloid.

Case Presentation

A 79-year-old man presented with bilateral leg swelling and was diagnosed with nephrotic syndrome with preserved renal function. Serum protein electrophoresis and immunofixation showed no measurable monoclonal protein. Serum lambda free light chains were elevated at 97.72 mg/L, and kappa:lambda ratio was 0.22. Pathology from renal biopsy showed focal deposits of Congo red staining. Liquid chromatography tandem mass spectrometry identified both fibrillogenic ApoIV signal sequence peptides and a peptide profile consistent with AL amyloid deposition.

Further testing showed mild Bence Jones proteinuria, mildly elevated beta-2 microglobulin, elevated cardiac troponin T, normal NT-pro-B-type natriuretic peptide, and normal Factor X activity. Echocardiogram and FDG PET CT were unremarkable. Bone marrow biopsy demonstrated a lambda-restricted monotypic plasma cell population comprising 10% of plasma cells by immunohistochemistry. Although the Congo red stain was indeterminate, IGH::CCND1 fusion was detected by FISH.

Treatment was initiated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone. Serum lambda light chains normalized after one cycle. Urine protein:creatinine ratio improved from 7.0 to 3.0 by the end of cycle 3. The patient will soon complete cycle 4 and undergo reassessment.

Discussion

While there are rare reports of AApoAIV renal amyloidosis and more prevalent cases of AL renal amyloidosis, the presence of both types of amyloid in the same patient has not been previously reported. AApoAIV renal amyloidosis has no established treatment and tends to present with rising serum creatinine as opposed to proteinuria. Given our patient’s clinical presentation with nephrotic syndrome and current light chain response to treatment, AL amyloidosis may be predominantly driving the disease.

Conclusions

Given the rarity of renal amyloidosis involving both AL and AApoAIV amyloid, the clinical course and optimal treatments are not established. The cumulative knowledge gained from individual case studies can serve as a basis for ongoing investigation and management of similar cases in the future.