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Camoriano, John

https://orcid.org/0000-0002-2957-5356

Mayo Clinic

1Mayo Clinic, Division of Hematology/Oncology, Department of Internal Medicine, Phoenix, United States

Citations 42

Predictors of symptom improvement in myeloproliferative neoplasms: A real-world retrospective cohort study
Khan, Muhammad Ali , Sarfraz, Muhammad Uzair, Sadiq, Muhammad Hussnain, Oberoi, Japneet Kaur, Camoriano, John , Yi, Cecilia Y. Arana , Blocher, Joshua, Palmer, Jeanne , Sarfraz, Muhammad Uzair, Sadiq, Muhammad Hussnain, Nahhat, Fouad , Oberoi, Japneet Kaur, Yi, Cecilia Arana , Andres, Jennifer , Kosiorek, Heidi , Riaz, Irbaz Bin

Blood | December 5, 2025 | Volume: 146 | Issue: Supplement 1 | Pages: 3823-3823

10.1182/blood-2025-3823 Get It

Abstract Introduction Persistent symptom burden in classic myeloproliferative neoplasms (MPNs) i.e., polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) is linked to shorter survival, more thromboembolic events and impaired quality of life. Identifying demographic, clinical, laboratory, and treatment-related predictors of symptom improvement can inform risk-adapted care and guide supportive interventions. Methods Patients with PV, ET, and MF were identified through a retrospective chart review. Total symptom scores (TSS) and scores of individual symptoms were recorded at the time of first TSS assessment. Demographic (age, sex, race, ethnicity), seasonal (summer: April–September; winter: October–March), clinical (time since diagnosis, depression, obesity, spleen size), treatment-related (phlebotomy, hydroxyurea, interferon-alpha, anagrelide, JAK inhibitors, antidepressants) and laboratory (complete blood counts, serum chemistry) factors were captured at the baseline. Symptom improvement was defined as a ≥50% TSS reduction from baseline. Cumulative incidence analyses, accounting for death as a competing risk, were performed to estimate the probability and timing of symptom improvement. Multicollinearity was assessed using variance inflation factors (VIFs). Highly correlated (VIF ≥5) features and features with ≥15% missing observations were excluded. Multivariable Fine-Gray hazards regression was performed to identify predictors of symptom improvement. Sub-distribution hazard ratios (sHR) with 95% confidence intervals (CI) were computed with p-values <0.05, indicating a statistically significant association with symptom improvement. Results A total of 532 patients (PV: 195, ET: 180, MF: 157) were identified between 2019 and 2024. The median age was 65 (IQR: 57-74) for PV, 65 (52-75) for ET, and 69 (63-75) for MF. Most patients were White (PV: 178, ET: 164, MF: 145) and female (PV: 99, ET: 134, MF: 84). Fatigue was the most frequently reported symptom (PV: 161, ET: 155, MF: 136) whereas weight loss was the least reported (PV: 9, ET: 12, MF: 26). Baseline TSS was highest in MF (median: 15; IQR: 7-27) followed by PV (13; 6-25) and ET (13; 5-23). Symptom improvement was observed in 89 (46%) PV, 71 (39%) ET, and 44 (28%) MF patients. Among those who improved, the cumulative incidence of symptom improvement reached 100% by 55 months in PV, 53 months in ET, and 58 months in MF. At 6, 12, and 24 months, cumulative symptom improvement was 22%, 34%, and 57% in PV; 15%, 25%, and 43% in ET; and 19%, 28%, and 39% in MF. Median time to improvement was not reached in any group. Death before symptom improvement was observed in 5 (3%) PV, 6 (3%) ET, and 37 (24%) MF patients. Multivariable regression analyses in PV identified higher baseline TSS (sHR: 1.02; 95%CI: 1.01-1.03) as associated with symptom improvement (p = 0.02). In ET, lower white blood cell count (sHR: 0.89; 95%CI: 0.81-0.98) and less time since diagnosis (0.99; 0.98-0.99) were associated with symptom improvement (p = 0.02). In MF, more months since diagnosis (sHR: 1.01 95%CI: 1.01-1.01) was associated with symptom improvement (p = 0.01). Conclusions Symptom trajectories differed across MPN subtypes. In PV, a higher baseline symptom burden; in ET, a lower white blood cell count and more recent diagnosis and in MF, a longer disease duration were associated with symptom improvement. These readily available parameters may help inform supportive strategies, tailor response expectations, and identify patients for symptom-directed interventions or clinical trials.
Predictors of Symptom Scores in Myeloproliferative Neoplasms: A Real‐World Retrospective Cohort Study
Khan, Muhammad Ali , Naqvi, Syed Arsalan Ahmed , Camoriano, John , Yi, Cecilia Y. Arana , Andres, Jennifer T., Blocher, Joshua L., Palmer, Jeanne , Camoriano, John K, Arana Yi, Cecilia Y , Riaz, Irbaz Bin , Palmer, Jeanne M , Khan, Muhammad Ali , Yi, Cecilia Arana , Andres, Jennifer T., Blocher, Joshua, Kosiorek, Heidi

Cancer Medicine | November 29, 2025 | Volume: 14 | Issue: 23 | Pages: No page available

10.1002/cam4.71333 Get It

ABSTRACT Background Symptom management in myeloproliferative neoplasms (MPN) remains challenging despite advancements in disease‐directed therapies. This study assessed the impact of demographic, clinical, laboratory and treatment‐related variables on total symptom scores (TSS) and individual symptom scores in patients with polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). Methods A cohort of 553 patients (PV: 200; ET: 190; MF: 163) was identified through a retrospective chart review. Symptom scores, demographic information and clinical variables were captured at the time of the first TSS assessment. Laboratory results within 1 month and treatment information within 90 days prior to the symptom assessment were captured. Univariable linear regression, followed by multivariable regression analyses using a robust variance estimator were performed, with a p value < 0.05 indicating a significant association. Results MF patients experienced the highest symptom burden and fatigue was the most reported symptom across all MPN subtypes. In PV, depression ( β : 10.53; p = 0.001) was associated with a higher TSS whereas older age (−0.17; 0.01) and higher hemoglobin (−1.24; 0.003) were associated with a lower TSS. In ET, depression ( β : 14.19; p < 0.001) was associated with a higher TSS, whereas in MF, depression (12.28; < 0.001) and higher WBC count (0.22; 0.02) were associated with a higher TSS. Depression and non‐White race in PV, depression in ET, and depression, low hemoglobin, and higher WBC count in MF were associated with multiple symptoms. Conclusions Integrating depression screening and management and optimizing hematologic parameters alongside disease‐directed therapy is crucial to improving patient outcomes.
Integrative Hematology Oncology Fellowship: A Descriptive Analysis of the First Accredited Program in the United States
Gowin, Krisstina , Larsen, Ashley , Camoriano, John

Blood | December 6, 2024 | Volume: 144 | Issue: Supplement 1 | Pages: 3826-3826

10.1182/blood-2024-211011 Get It

Introduction: The presence of integrative oncology programs in cancer care institutions is rapidly increasing globally, however specialized training in integrative oncology is still limited. In 2022, we launched the first Integrative Medicine in Hematology Oncology (IMHO) fellowship within a traditional Accreditation Council for Graduate Medical Education (ACGME) Hematology Oncology Fellowship in the US. In 2024, the fellowship received accreditation from the Academic Consortium for Integrative Medicine and Health. Here we describe the educational model and report on the fellowship experience via qualitative analysis. Methods: Fellows are recruited from Hematology Oncology Fellows at University of Arizona and Mayo Clinic Arizona. A total of 1000+ hours of Integrative medicine curriculum is completed over 3-years to fulfill American Board of Integrative Medicine requirements for board eligibility. Curriculum is a hybrid delivered via The Andrew Weil Center for Integrative Medicine Foundational Fellowship Curriculum (550 hours), dedicated Integrative Oncology curriculum of didactic and clinical training (250 hours), and research training and investigator-initiated project (200 hours).. Didactic and clinical compentecy-based assessments are required for programmatic completion. Interview-based assessments of acceptability are conducted annually throughout the fellowship duration. Results: IMHO Model Acceptability: Successful recruitment occurred annually since the program's inception (2022, 2023, 2024) of at least 2 fellows of 8 traditional ACGME matched fellows. Additionally, ACGME fellows expressed interest in IMHO fellowship exceeding IMHO recruitment goals. The optimal year of start for IMHO fellowship was determined to be post-graduate year 4 (PGY-4). Five of seven fellows met research goal milestones, and it was determined robust mentorship and research should also start in PGY-4 year. Incentives and cohort visible “dashboard” of progress was deemed necessary when online curriculum milestones were not routinely met by fellows. We received accreditation from the Academic Consortium for Integrative Medicine and Health in spring of 2024. Our first fellow graduate completed requirements in 2024 and is board-eligible in Hematology, Oncology, and Integrative Medicine. Qualitative Analysis: Fellows actively participating in the IMHO fellowship program participated in focus groups. Common themes included: 1) application of resiliency building applied to fellows lives personally and 2) providing tools that allowed them to incorporate integrative medicine to enhance the overall care of their patients. One Fellow stated, “I don't regret picking this choice (the IMHO Fellowship) because I have seen that from my own experience with the patients that I've been able to help them throughout their journey with cancer ...using aspects of Integrative Medicine.” In applying the tools clinically another stated, “I feel like we're really trying to treat the person as a whole, not just treating their condition, and we're really emphasizing their overall goals in life beyond just whatever diagnosis that they have.” One fellow pointed out in regard to their own resiliency “the fellowship program has actually helped improve my resilience and my own wellness ”and “I think it's a really good foundation to have for the future” and another stated “I think it helps me not fall off track because it's a constant reminder of things that you're already interested in, and things that you believe in, that are good coping mechanisms and good ways to kind of, find a balance in your life with your own wellbeing and the wellbeing of others around you.” Finally, “I would say that from my experience I think it's made me a well-rounded physician.” Conclusion: The IMHO Fellowship is the first accreditated Integrative Hematology Oncology training program in the nation. We found the IMHO model an acceptable and desirable model of Integrative Oncology education. Our program revealed optimal timing of initiation is the PGY-4 year, incentive and productivity dashboards are needed for online learning productivity, and research productivity is contingent on robust mentorship and productivity milestones. Ongoing assessment is necessary to continue to optimize the IMHO fellowship model.
Predictors of Individual Symptom Scores in Myeloproliferative Neoplasms: A Real-World Retrospective Cohort Study
Khan, Muhammad Ali , Naqvi, Syed Arsalan Ahmed , Yi, Cecilia Y. Arana , Camoriano, John , Blocher, Joshua, Palmer, Jeanne , Yi, Cecilia Arana , Andres, Jennifer , Riaz, Irbaz Bin

Blood | December 5, 2024 | Volume: 144 | Issue: Supplement 1 | Pages: 5195-5195

10.1182/blood-2024-212240 Get It

Introduction Symptom burden in myeloproliferative neoplasms (MPN) is quantified using total symptom scores (TSS) calculated from the patient-reported scores for clinically meaningful symptoms. The impact of demographic, clinical, and laboratory features on these symptoms remains unclear and warrants further investigation. Methods Patients with polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF) were identified from the retrospective chart review. TSS, scores of individual symptoms (fatigue, early satiety, abdominal discomfort, inactivity, concentration difficulties, fever, night sweats, itching, bone pain, weight loss), demographic characteristics (race, ethnicity, age, and gender), clinical features (time since diagnosis, depression status, obesity status), laboratory results and season at the time of visit when the first TSS assessment was performed were recorded. Multicollinearity and homoscedasticity were assessed using variance inflation factors and visual inspection of the data, respectively. A multivariable regression analysis was performed using a robust variance estimator. A p-value &lt;0.05 indicated a statistically significant association with the symptom scores. Results The chart review identified 557 (PV: 201; ET: 191; MF: 165) patients. The median age of patients with PV, ET, and MF was 65 (range: 27-93), 65 (20-96), and 69 (27-96) years, respectively. Most patients were white (PV: 92%; ET: 90%; MF: 93%) and non-Hispanic or Latino (PV: 93%; ET: 96%; MF: 92%). Most patients with PV and ET were female (PV: 51%; ET: 73%), while most patients with MF were male (55%). Among the included patients 22% (PV: 21%; ET: 27%; MF: 18%) had the diagnosis of depression at the time of the clinical visit. TSS was highest in MF (mean (SD): 18.1±14.6), followed by PV (15.7±14.5) and ET (15.1±14.0) patients. Fatigue was the most reported symptom by the patients (PV: 81%; ET: 82%; MF: 84%), whereas weight loss was the least reported symptom (PV: 4.5%; ET: 6.3%; MF: 16%). With regards to the TSS multivariable regression analysis showed that age (beta coefficient: 0.2; p=0.01), depression (7.2; 0.008), and low hemoglobin (1.2; 0.003) in PV, depression (11.7; 0.001) in ET, and depression (9.5; 0.001), splenomegaly (6.9; 0.01), and WBC count (0.16; 0.048) in MF patients were significantly associated with TSS. In terms of individual symptoms in PV, depression was associated with fatigue (beta coefficient: 1.31; p-value: 0.01) and concentration difficulties (1.7; &lt;0.001), high WBC count was associated with early satiety (0.09; 0.03), and non-white race was associated with fever (1.4; 0.007). In ET, depression was associated with fatigue (1.6; &lt;0.001), abdominal discomfort (1.4; &lt;0.001), inactivity (2.5; &lt;0.001), concentration difficulties (2.4; &lt;0.001) and night sweats (1.4; &lt;0.001), anemia was associated with fatigue (1.2; 0.03), fewer months since diagnosis was associated with inactivity (0.01; 0.02) and young age was associated with bone pain (0.02; 0.03). In MF, depression was associated with fatigue (1.8; 0.001), concentration difficulties (1.3; 0.01), fever (1.1; 0.02), and night sweats (2.2; 0.03), low hemoglobin was associated with fatigue (0.46; &lt;0,001) and early satiety (0.31; 0.003), high WBC count was associated with fatigue (0.03; 0.04) and early satiety (0.04; 0.02), low platelet count was associated with abdominal discomfort (0.002; 0.03), splenomegaly was associated with inactivity (2.27; 0.003) and female gender was associated with itching (0.57; 0.01). Conclusions This study demonstrates that depression in PV and ET and depression, low hemoglobin, and a high WBC count in MF contribute to multiple symptoms seen in these patients. Identifying demographic groups at risk of a higher symptom burden and addressing the comorbidities that drive symptoms, in addition to disease-directed therapy, is crucial to improving the quality of life in these patients.
Patient Cost Savings When Using Telehealth for Management of Myeloproliferative Neoplasms
Khan, Muhammad Ali , Naqvi, Syed Arsalan Ahmed , Yi, Cecilia Y. Arana , Camoriano, John , Padrnos, Leslie , Blocher, Joshua, Palmer, Jeanne , Yi, Cecilia Arana , Andres, Jennifer , Riaz, Irbaz Bin

Blood | December 1, 2023 | Volume: 142 | Issue: Supplement 1 | Pages: 6409-6409

10.1182/blood-2023-187931 Get It

Introduction: Telemedicine has been widely utilized in the healthcare marketplace since the beginning of the COVID-19 pandemic. Over the last 3 years, virtual care has been widely employed in the care of patients with polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) at Mayo Clinic. These rare disorders have few experts; therefore, telemedicine becomes an optimal approach for patients receive expert care even when they are not close to the medical center. 1 Additionally, telemedicine reduces financial burden for the patients. We aim to estimate potential cost savings to patients utilizing telemedicine. Methods: We identified patients with MPN seen at Mayo Clinic, Arizona who have had at least 2 visits (1 or more virtual visits) between March 2020 and June 2022. We extracted retrospective data from electronic medical records (EMR) including sociodemographic data, disease related data and number of visits - both virtual and in person - of the patients identified. The address recorded from the EMR was assumed to be the patient's location at the time of virtual visit. Driving distances were determined using zip codes in Google Maps and the patients were stratified into quartiles based on round trip travel distances. Patients in quartiles 1 through 3 were assumed to have driven while patients in quartile 4 were assumed to have traveled by plane. Patients in quartile 1 were assumed to have 1 travel day including the follow-up visit and no accommodation, patients in quartile 2 were assumed to have 2 travel days including the follow up visit and 1 night of accommodation whereas patients in quartiles 3 and 4 were assumed to have 2 travel days, 1 day for the follow up visit and 2 nights of accommodation. Mileage rate costs were based on US Internal Revenue Service (IRS) standard mileage rates for medical or moving purposes for 2021. 2 Owing to extreme variability in flight costs, flying costs were assumed to be equal to the driving costs. Per diem costs for accommodation and meals and incidentals were based on the US General Services Administration (GSA) 2021 rates for Phoenix 3 with the cost being 75% of the full per diem rate on traveling days via GSA formulaic approach. Results: One hundred and eight patients having 282 virtual visits were identified. Patients ranged from 30 - 87 years with the mean and median age of 65 years and 67 years respectively. Majority of the patients were female (62%) and white (98%) with gender distribution varying by distance. Descriptive statistics of the estimated out-of-pocket costs for patients by distance quartiles are summarized in Table 1a. Mean estimated cost savings ranged from $46.8 to $877.3 with the savings of $43.8 - $1372.2 at extremes. The total cost savings by all patients was $90,306.7. The breakdown of costs by category for each quartile is shown in Table 1b which shows that the majority of costs saved by patients in quartile 1 were due to avoiding costs spent on meals and incidentals (~90% of mean costs) whereas costs saved due to meals and incidentals for patients in quartile 4 were only ~16% of the mean costs. Conclusions: Our research highlighted telemedicine as a cost-effective approach for patients with MPN who received virtual care from March 2020 - June 2022. Study limitations included not weighing costs for childcare or accompanying individuals and using driving costs instead of flying costs, however, these considerations potentially underestimate the actual cost savings to the patient. Additionally, since the median age of this population was 65, we didn't include loss of wages to the analysis, which may further underestimate the costs. Despite concerns over billing, reimbursement, and state licensure, virtual care is being established as a critical part of medicine moving forward. 1. Doolittle, G.C. and A.O. Spaulding, Providing Access to Oncology Care for Rural Patients via Telemedicine. Journal of Oncology Practice, 2006. 2(5): p. 228-230. 2. Internal Revenue Service. Standard mileage rates for 2021. Available at: https://www.irs.gov/tax-professionals/standard-mileage-rates 3. US General Services Administration. Per diem rates. Available at: https://www.gsa.gov/travel/plan-book/per-diem-rates/per-diem-rates-results?action=perdiems_report&fiscal_year=2021&state=AZ&city=&zip=85054
Integrative Hematology Oncology Fellowship Program: A National Survey Based Analysis of Need
Gowin, Krisstina , Wertheim, Betsy C. , Larsen, Ashley , Camoriano, John

Blood | November 23, 2021 | Volume: 138 | Issue: Supplement 1 | Pages: 1887-1887

10.1182/blood-2021-151413 Get It

Abstract Introduction: Integrative hematology oncology (IHO), the combination of complementary medicine in conjunction with conventional cancer treatments, is emerging as an important supportive care modality. Formal education programs, such as IHO fellowship programs, are not currently available. We conducted a national survey to gauge interest and investigate barriers to the development of IHO training programs in the US. Methods: An 18-question survey was sent to hematology/oncology, radiation oncology, and palliative care program directors, physicians, and fellows, and internal medicine residents planning to enter into any of the above fellowship programs. Recruitment was national with use of public email list servs and social media. Program directors and fellowship programs were identified through the Accreditation Council for Graduate Medical Education (ACGME). Participant informed consent was completed online and, Qualtrics survey links were shared weekly via email in two 4-week sessions. Results: Participants (n=208) included physicians from: Hematology/Oncology 65.9% (n=137), Radiation Oncology 8.6%(n=18) and Palliative Care 25.4% (n=53). Hematology oncology respondents included physicians 47.4% (n=65), fellows 33.6% (n=46), and residents planning to enter into a hematology oncology fellowship 3.6% (n=5). Program directors (n=69) from each specialty included: 15.3% (n=21) from hematology/oncology, 61.1% (n=11) from radiation oncology, and 69.8% (n=37) from palliative care. Interestingly, palliative care had the highest response rate in this category. Survey respondents were mostly aged 31-40 y (38.4%), 41-50 y (23.8%), or 51-60 y (21.5%). Participant genders were male (45.7%), female (50.9%), or prefer not to identify (3.5%). There was diverse regional participation across the United States. Overall participant responses revealed significant interest for an IHO training program, despite little (36.0%) to no (49.4%) previous training in integrative medicine (Table 1). Curriculum topics of primary interest were physician/provider wellness, stress reduction techniques, off-label use of medication, exercise/physical activity, nutrition, and lifestyle counseling. The preferred education delivery was didactic lectures and clinical practice exposure. Barriers to implementation included lack of good curriculum and inadequate budget/time/faculty experience to implement the program. Need for IHO training was based on perceived benefit to patients in 39 participants (73.6%), and patient request for services in 37 participants (68.8%). Conclusion: In this national survey of 208 physicians, the majority (64%) expressed interest in IHO education programs. Barriers included lack of curriculum, expertise, budget, and time. Education preference for fellowship track and certificate programs were similar, suggesting interest of both available options. Most participants (87%) felt fellows should participate in IHO research. In conclusion, IHO training is perceived as valuable and desirable. Thus, the development and dissemination of IHO training programs is needed. Figure 1 Figure 1. Disclosures Gowin: Incyte: Speakers Bureau.
Relapsed Refractory Acquired Thrombotic Thrombocytopenic Purpura (aTTP) Following COVID-19 Vaccination
Francisco, Michael T., Kaufman, Adrienne, Northfelt, Donald W. , Padrnos, Leslie , Adamski, Jill , Kinard, Theresa N. , Camoriano, John , Rosenthal, Allison , Andres, Jennifer

Blood | November 23, 2021 | Volume: 138 | Issue: Supplement 1 | Pages: 4218-4218

10.1182/blood-2021-151964 Get It

Abstract Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) due to an acquired deficiency in the enzyme ADAMTS13 leads to ultra-large von Willebrand multimers, thrombocytopenia and microangiopathic hemolytic anemia. Complications include microvascular and macrovascular thrombosis. We present an unusual case of a patient with a history of refractory aTTP who experienced relapsed aTTP following COVID-19 vaccine. Case Description: A 57-year-old African-American male with a history of refractory aTTP experienced a relapse following 3 years of remission after receiving COVID-19 vaccination. The patient was initially diagnosed with aTTP in 2016, after presenting with symptoms of dark urine, mild headaches and transient episodes of aphasia and paresthesia. Due to symptoms and persistently low ADAMTS13 levels, he required prolonged and extensive treatment including over 5 weeks of daily therapeutic plasma exchange (TPE), followed by gradual reduction in frequency of TPE sessions, as well as trials of rituximab, eculizumab, steroids, mycophenolate mofetil and bortezomib. Ultimately, he achieved remission after 9 months of intermittent TPE, 3 months of weekly bortezomib 1 mg/m 2, mycophenolate mofetil up-titrated to 1,750 mg twice daily, and then slowly tapered off over a 2-year period. The patient was doing well for 3 years without manifestations of aTTP (2 years off all therapeutics), until he developed a petechial rash 7 weeks after receiving the second dose of the Moderna COVID-19 vaccine. He was found to have acute thrombocytopenia with platelets of 38 x 10 9/L (normal range 135-317 x 109/L), from a baseline of 200-300 x 10 9/L. He was referred to the emergency department, where additional labs were notable for mildly elevated LDH of 508 U/L (normal range 122-222 U/L), hemoglobin of 12.4 g/dL (normal range 13.2-16.6 g/dL), creatinine at baseline, and peripheral blood smear showing 1-3 schistocytes per high-powered field. ADAMTS13 activity level was t &lt;5% (normal &gt;/= 70%), with positive ADAMTS13 inhibitor screen and titer of 1.5 (normal &lt;0.4), consistent with relapsed aTTP. The patient was admitted to the hospital, and initiated on daily TPE, with steroids and diphenhydramine prior to each TPE session. He quickly improved with TPE alone , but given his history of refractory aTTP, he was discharged on weekly rituximab for 4 weeks and caplacizumab 11 mg daily for 30 days. His platelets remained stable within the upper limit of normal during his 30 day course of caplacizumab. However, 3 weeks after completion of caplacizumab, he had an acute drop in his platelets to 23 x 10 9/L. His ADAMTS13 level was again found to be &lt;5%, and inhibitor level was the highest that it had ever been at 11.4. He was again hospitalized and underwent 8 sessions of daily TPE, as well as re-initiation of caplacizumab, mycophenolate mofetil 500 mg bid (with increasing taper), and a prednisone taper. Intravenous Cyclophosphamide 750 mg/m 2 was also added every 3 weeks. With this regimen, patient's platelet count normalized and remain stable, and his ADAMTS13 activity level has reached 52-59%. Discussion: Cases of vaccine-induced immune thrombotic thrombocytopenia (VITT) have been described as a complication following vaccination with formulations containing replication-defective adenoviral vectors (AstraZeneca-Oxford and Johnson&Johnson COVID-19 vaccines)(Arepally and Ortel 2021, Simpson, Shi et al. 2021). VITT and aTTP are both immune-mediated, however, VITT is distinct and pathogenically linked to autoimmune heparin-induced thrombocytopenia (HIT), given the presence of anti-platelet factor 4 antibodies in these patients, whereas aTTP is due to reduction in ADAMTS13 level, secondary to an antibody inhibitor of ADAMTS13 (Arepally and Ortel 2021). Recently, cases have been reported of de novo aTTP developing shortly after COVID-19 vaccination with all available vaccines, except the Moderna (mRNA-1273) vaccine (Al-Ahmad, Al-Rasheed et al. 2021, de Bruijn, Maes et al. 2021, Maayan, Kirgner et al. 2021, Ruhe, Schnetzke et al. 2021, Waqar, Khan et al. 2021, Yocum and Simon 2021). Additionally, cases of relapsed aTTP have been described following only the BNT162B2 (Pfizer-BioNTech) vaccine (Maayan, Kirgner et al. 2021, Sissa, Al-Khaffaf et al. 2021). This is the first case, to our knowledge, reported in the literature of aTTP following vaccination with Moderna's mRNA-1273 vaccine. Disclosures No relevant conflicts of interest to declare.
Pregnancy in patients with myelofibrosis: Mayo–Florence series of 24 pregnancies in 16 women
Gangat, Naseema , Guglielmelli, Paola , Al‐Kali, Aref , Wolanskyj, Alexandra P. , Camoriano, John , Patnaik, Mrinal M. , Pardanani, Animesh , Hanson, Curtis A. , Tefferi, Ayalew , Wolanskyj-Spinner, Alexandra P., Tefferi, Ayalew , Wolanskyj-Spinner, Alexandra P , Guglielmelli, Paola , Vannucchi, Alessandro M.

British Journal of Haematology | June 14, 2021 | Volume: 195 | Issue: 1 | Pages: 133-137

10.1111/bjh.17626 Get It

Ropeginterferon Demonstrates Safety and Efficacy in Myelofibrosis: Pilot Study Results
Palmer, Jeanne , Camoriano, John , Zimmerman, Craig, Kosiorek, Heidi , Dueck, Amylou C. , Mesa, Ruben A.

Blood | November 4, 2020 | Volume: 136 | Issue: Supplement 1 | Pages: 23-23

10.1182/blood-2020-138934 Get It

Background: Myelofibrosis (MF) is a clonal bone marrow disorder characterized by a constitutional symptoms, splenomegaly, anemia, elevated WBC and bone marrow fibrosis. Ruxolitinib is a JAK2 inhibitor approved for patients with Intermediate-2 (INT-2) or high risk disease based on dynamic international prognostic scoring system (DIPSS), however, there are limited options for patients with early disease. Interferon alpha (IFNa) is a cytokine therapy that has been used in treatment of chronic myelogenous leukemia and polycythemia vera (PV). In these diseases, there can be a reduction in the clonal burden in addition to a hematologic response. Therefore, we sought to use this therapy in patients with MF. Methods: Patients who had MF were enrolled on an IRB approved clinical protocol in a single arm, single institution study. Patients were stratified to either an early disease arm (characterized by intermediate 1 (INT-1) risk or advanced disease (Int-2 or high-risk disease based on DIPSS). Patients were started on ropeginterferon (interferon-alpha 2b) at 50 ug every two weeks, and the dose was titrated up to either 300 ug every other week, or until unacceptable toxicity. Patients were monitored for response based on IWG criteria. Results: Ten patients with MF have been enrolled on the study to date (Early: 3, Int/High: 7). Median age was 69.5 (39 - 88), four patients were female. Five patients had primary MF (PMF), 4 had post-essential thrombocythemia and 1 post PV MF. Five patients had previous treatment for their disease. Four patients continue to receive active treatment (2 early disease, and 2 advanced disease). Reasons for stopping include disease progression (3), toxicity (1), alternate treatment (1) and lack of benefit (1). The median number of cycles for those who ended treatment was 7.5 (4-47), and for those who remain on treatment, they range from 16 - 46 cycles. Five patients achieved a clinical improvement, 3 had a spleen response, and 2 had a symptom response. A grade 3 adverse event (AE) potentially attributable to the treatment occurred in 6 patients (60%), 4 (40.0%) patients had anemia, and 2 (20.0%) patients had lymphopenia. Only one patient discontinued therapy due to AEs (dizziness, atrial fibrillation). One death has occurred to date, due to an unrelated cause. Discussion: Ropeginterferon is a well-tolerated therapy that results in clinical improvement in half the patients, and appears to be well tolerated. These results, along with historic data suggesting possible reduction in clonal burden, suggest that ongoing studies are warranted to further understand the clinical benefit of ropeginterferon. Disclosures Zimmerman: Pharmaessentia: Current Employment. Mesa:Incyte: Research Funding; AbbVie: Research Funding; Sierra Oncology: Consultancy; CTI BioPharma: Research Funding; Promedior: Research Funding; Genentech: Research Funding; Novartis: Consultancy; Samus Therapeutics: Research Funding; LaJolla Pharmaceutical Company: Consultancy; Bristol Myers Squibb: Research Funding. OffLabel Disclosure: Clinical trial