Perioperative management of myeloproliferative neoplasms: A <scp>pan‐Canadian</scp> physician survey and international expert opinion

Szuber, Natasha; Toliopoulos, Panagiota; Busque, Lambert; Cerquozzi, Sonia; Foltz, Lynda; Gupta, Vikas; Tefferi, Ayalew; Vannucchi, Alessandro M.; Hillis, Christopher; Leber, Brian; Maze, Dawn; Prchal, Jaroslav F.; Olney, Harold J.; Sirhan, Shireen

doi:10.1002/ajh.26739

Cited by 4 publications

(2 citation statements)

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“…More recent studies have suggested more favorable outcomes; in one such study, 178 255 patients with PV or ET were analyzed for a total of 311 surgical interventions, including 25 emergency procedures; antithrombotic prophylaxis included subcutaneous heparin in 54% and antiplatelet therapy in 15% of the patients; in addition, 74% of patients were on cytoreductive therapy before surgery; the 3‐month postoperative course was uneventful in more than 80% of the cases whereas arterial or venous events were documented in 12 patients, each, with the former being more frequent in ET and the latter in PV; major bleeding complications occurred in 23 cases and deaths in 5; platelet count and hematocrit level at the time of surgery were not predictive of vascular events and the value of pre‐procedure prophylactic therapy was not apparent. The lack of a standardized approach to perioperative care in patients with myeloproliferative neoplasms was recently underlined by a pan‐Canadian physician survey 179 …”

Section: Treatmentmentioning

confidence: 99%

Polycythemia vera: 2024 update on diagnosis, risk‐stratification, and management

Tefferi

Barbui

2023

American J Hematol

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Disease Overview Polycythemia vera (PV) is a JAK2‐mutated myeloproliferative neoplasm characterized by clonal erythrocytosis; other features include leukocytosis, thrombocytosis, splenomegaly, pruritus, constitutional symptoms, microcirculatory disturbances, and increased risk of thrombosis and progression into myelofibrosis (post‐PV MF) or acute myeloid leukemia (AML). Diagnosis A working diagnosis is considered in the presence of a JAK2 mutation associated with hemoglobin/hematocrit levels of >16.5 g/dL/49% in men or 16 g/dL/48% in women; morphologic confirmation by bone marrow examination is advised but not mandated. Cytogenetics Abnormal karyotype is seen in 15%–20% of patients with the most frequent sole abnormalities being +9 (5%), loss of chromosome Y (4%), +8 (3%), and 20q− (3%). Mutations Over 50% of patients harbor DNA sequence variants/mutations other than JAK2, with the most frequent being TET2 (18%) and ASXL1 (15%). Prognostically adverse mutations include SRSF2, IDH2, RUNX1, and U2AF1, with a combined incidence of 5%–10%. Survival and Prognosis Median survival is ⁓15 years but exceeds 35 years for patients aged ≤40 years. Risk factors for survival include older age, leukocytosis, abnormal karyotype, and the presence of adverse mutations. Twenty‐year risk for thrombosis, post‐PV MF, or AML are ⁓26%, 16% and 4%, respectively. Risk Factors for Thrombosis Two risk categories are considered: high (age >60 years or thrombosis history) and low (absence of both risk factors). Additional predictors for arterial thrombosis include cardiovascular risk factors and for venous thrombosis higher absolute neutrophil count and JAK2V617F allele burden. Treatment Current goal of therapy is to prevent thrombosis. Periodic phlebotomy, with a hematocrit target of <45%, combined with once‐ or twice‐daily aspirin (81 mg) therapy, absent contraindications, is the backbone of treatment in all patients, regardless of risk category. Cytoreductive therapy is reserved for high‐risk disease with first‐line drugs of choice being hydroxyurea and pegylated interferon‐α and second‐line busulfan and ruxolitinib. In addition, systemic anticoagulation is advised in patients with venous thrombosis history. Additional Treatment Considerations At the present time, we do not consider a drug‐induced reduction in JAK2V617F allele burden, which is often incomplete and seen not only with peg‐IFN but also with ruxolitinib and busulfan, as an indicator of disease‐modifying activity, unless accompanied by cytogenetic and independently‐verified morphologic remission. Accordingly, we do not use the specific parameter to influence treatment choices. The current review also includes specific treatment strategies in the context of pregnancy, splanchnic vein thrombosis, pruritus, perioperative care, and post‐PV MF.

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Section: Treatmentmentioning

confidence: 99%

Polycythemia vera: 2024 update on diagnosis, risk‐stratification, and management

Tefferi

Barbui

2023

American J Hematol

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“…More recent studies have suggested more favorable outcome; in one such study, 153 255 patients with PV or ET were analyzed for a total of 311 surgical interventions, including 25 emergency procedures; antithrombotic prophylaxis included subcutaneous heparin in 54% and antiplatelet therapy in 15% of the patients; in addition, 74% of patients were on cytoreductive therapy before surgery; 3‐month post‐operative course was uneventful in more than 80% of the cases, whereas arterial or venous events were documented in 12 patients, each, with the former being more frequent in ET and the latter in PV; major bleeding complications occurred in 23 cases and deaths in 5; platelet count and hematocrit level at time of surgery were not predictive of vascular events and the value of pre‐procedure prophylactic therapy was not apparent. The lack of a standardized approach to perioperative care in patients with MPN was recently underlined by a pan‐Canadian physician survey 154 . Our current practice regarding perioperative management in ET is based more on intuition rather than evidence and includes keeping platelet count below 450 × 10 9 /L, before and at least 1 week after surgery; platelet count control in low‐risk patients might require a short course of treatment with HU; in addition to cytoreductive therapy, careful use of LMWH is advised in high‐risk patients.…”

Section: Treatment Strategies In Specific Contextsmentioning

confidence: 99%

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management

Tefferi,

Vannucchi,

Barbui

2024

American J Hematol

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OverviewEssential thrombocythemia is a Janus kinase 2 (JAK2) mutation‐prevalent myeloproliferative neoplasm characterized by clonal thrombocytosis; clinical course is often indolent but might be interrupted by thrombotic or hemorrhagic complications, microcirculatory symptoms (e.g., headaches, lightheadedness, and acral paresthesias), and, less frequently, by disease transformation into myelofibrosis (MF) or acute myeloid leukemia.DiagnosisIn addition to thrombocytosis (platelets ≥450 × 109/L), formal diagnosis requires the exclusion of other myeloid neoplasms, including prefibrotic MF, polycythemia vera, chronic myeloid leukemia, and myelodysplastic syndromes with ring sideroblasts and thrombocytosis. Bone marrow morphology typically shows increased number of mature‐appearing megakaryocytes distributed in loose clusters.Genetics: Approximately 80% of patients express myeloproliferative neoplasm driver mutations (JAK2, CALR, MPL), in a mutually exclusive manner; in addition, about 50% harbor other mutations, the most frequent being TET2 (9%–11%), ASXL1 (7%–20%), DNMT3A (7%), and SF3B1 (5%). Abnormal karyotype is seen in <10% of patients and includes +9/20q−/13q‐.Survival and PrognosisLife expectancy is less than that of the control population. Median survival is approximately 18 years but exceeds >35 years in younger patients. The triple A survival risk model, based on Age, Absolute neutrophil count, and Absolute lymphocyte count, effectively delineates high‐, intermediate‐1‐, intermediate‐2‐, and low‐risk disease with corresponding median survivals of 8, 14, 21, and 47 years.Risk Factors for ThrombosisFour risk categories are considered: very low (age ≤60 years, no thrombosis history, JAK2 wild‐type), low (same as very low but JAK2 mutation present), intermediate (same as low but age >60 years), and high (thrombosis history or age >60 years with JAK2 mutation).Mutations and PrognosisMPL and CALR‐1 mutations have been associated with increased risk of MF transformation; spliceosome with inferior overall and MF‐free survival; TP53 with leukemic transformation, and JAK2V617F with thrombosis. Leukemic transformation rate at 10 years is <1% but might be higher in JAK2‐mutated patients with extreme thrombocytosis and those with abnormal karyotype.TreatmentThe main goal of therapy is to prevent thrombosis. In this regard, once‐daily low‐dose aspirin is advised for all patients and twice daily for low‐risk disease. Cytoreductive therapy is advised for high‐risk and optional for intermediate‐risk disease. First‐line cytoreductive drugs of choice are hydroxyurea and pegylated interferon‐α and second‐line busulfan.Additional ContentThe current review includes specific treatment strategies in the context of extreme thrombocytosis, pregnancy, splanchnic vein thrombosis, perioperative care, and post‐essential thrombocythemia MF, as well as new investigational drugs.

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Perioperative outcomes in patients with myeloproliferative neoplasms: a multicentric analysis of 354 surgical procedures

Szuber,

Olney,

Dagenais Bellefeuille

et al. 2024

Blood Vessels, Thrombosis & Hemostasis

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Perioperative management of myeloproliferative neoplasms: A pan‐Canadian physician survey and international expert opinion

Cited by 4 publications

References 11 publications

Polycythemia vera: 2024 update on diagnosis, risk‐stratification, and management

Polycythemia vera: 2024 update on diagnosis, risk‐stratification, and management

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management

Perioperative outcomes in patients with myeloproliferative neoplasms: a multicentric analysis of 354 surgical procedures

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