<scp>ABO</scp> blood group type and risk of venous thrombosis in essential thrombocythemia

Karrar, Omer; Abdelmagid, Maymona; Vannucchi, Alessandro M.; Barbui, Tiziano; Tefferi, Ayalew; Gangat, Naseema

doi:10.1111/bjh.18906

Cited by 5 publications

(5 citation statements)

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“…69 Separate analysis of arterial and venous thrombosis was performed on the IPSET database of 891 patients with WHO-defined ET; 70 both arterial and venous thrombotic events, in the Mayo cohort, while a similar protective role was apparent for cytoreductive therapy, in the Florence cohort. Taken together, these observations generally support our current antithrombotic treatment approach in ET (detailed in the next section) [73][74][75] with room for improvement in both event prediction 63,72,[76][77][78] and optimal prevention of thrombosis. 79,80 Extreme thrombocytosis, operationally defined as a platelet count of ≥1000 Â 10 9 /L, has been associated with acquired von Willebrand syndrome (AvWS) and excess bleeding, especially in the presence of aspirin therapy.…”

Section: Risk Factors For Thrombosis and Bleedingsupporting

confidence: 67%

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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Section: Risk Factors For Thrombosis and Bleedingsupporting

confidence: 67%

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

Tefferi,

Vannucchi,

Barbui

2024

American J Hematol

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“…As we already mentioned, a significant proportion of ET patients with B-type blood group in the study by Karrar et al [1] were treated with cytoreduction (83%), antiplatelet (73%), and anticoagulant treatment (48%), calling for other possible explanations for the increased thrombotic risk in this particular subset of patients. Considering that several prior studies have already demonstrated an increased risk of venous thromboembolism in persons with non-O blood types [8][9][10],…”

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confidence: 88%

“…

With great interest, we have read the recent article by Karrar et al [1], published in the British Journal of Haematology, which investigated the impact of blood group type on clinical outcomes in patients with essential thrombocythemia (ET). The authors have reported that patients with B-type blood group were more prone to venous thrombosis than patients with other blood groups; this effect was independent of antiplatelet and cytoreductive treatment and was confined to males.We would like to point out that even though ET patients with B-type blood group more frequently received cytoreductive and anticoagulant treatment in comparison to patients with other blood groups (Table 1 in the original article), they suffered from more venous events [1].This interesting observation deserves a comment and may suggest that other underlying pathophysiological mechanisms could be responsible for the reported phenomenon.

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confidence: 99%

“…With great interest, we have read the recent article by Karrar et al [1], published in the British Journal of Haematology, which investigated the impact of blood group type on clinical outcomes in patients with essential thrombocythemia (ET). The authors have reported that patients with B-type blood group were more prone to venous thrombosis than patients with other blood groups; this effect was independent of antiplatelet and cytoreductive treatment and was confined to males.…”

mentioning

confidence: 99%

“…we would like to suggest to the authors to analyze whether DARCnegative phenotype may have been associated with higher thrombotic risk in their study [1]. As MPNs are characterized by constitutive leukocyte activation and chronic inflammatory state which are both, at least partly, responsible for the increased thrombotic risk [11,12], this particular erythrocyte phenotype may accentuate inflammation and increase leukocyte trafficking through vascular endothelium thus having the potential to promote thrombosis in all ET patients not only in those with B-type blood group.…”

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confidence: 99%

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