bl-4162a and Venous-Thromboembolism

Myeloproliferative neoplasms (MPNs) share common clonal stem cells but show significant differences in their clinical courses. The aim of this retrospective study was to evaluate thrombotic and hemorrhagic complications, JAK2 status, gastrointestinal and cardiac changes, treatment modalities, and survival in MPNs in Turkish patients.. Medical files of 294 patients [112 essential thrombocythemia (ET), 117 polycythemia vera (PV), 46 primary myelofibrosis, and 19 unclassified MPN cases] from 2 different universities in Turkey were examined.. Older age, higher leukocyte count at diagnosis, and JAK2 mutation positivity were risk factors for thrombosis. Platelet count over 1000x109/L was a risk factor for hemorrhagic episodes. Hydroxyurea treatment was not related to leukemic transformation. Median follow-up time was 50 months (quartiles: 22.2-81.75) in these patients. Patients with primary myelofibrosis had the shortest survival of 137 months when compared with 179 months for ET and 231 months for PV. Leukemic transformation, thromboembolic events, age over 60 years, and anemia were found to be the factors affecting survival.. Thromboembolic complications are the most important preventable risk factors for morbidity and mortality in MPNs. Drug management in MPNs is done according to hemoglobin and platelet counts. Based on the current study population our results support the idea that leukocytosis and JAK2 positivity are more important risk factors for thrombosis than hemoglobin and platelet values.. Amaç: Miyeloproliferatif hastalıklar (MPH) ortak klonal bir kök hücreden köken almalarına karşın klinik seyirleri belirgin farklılıklar göstermektedir. Bu retrospektif çalışmanın amacı MPH’lardaki trombotik ve hemorajik komplikasyonların, JAK2 mutasyon durumunun, gastrointestinal ve kardiyak değişikliklerin, tedavi şekillerinin ve yaşam sürelerinin incelenmesidir. Gereç ve Yöntemler: Türkiye’nin iki farklı üniversite hastanesinden 294 hastanın [112 esansiyel trombositemi (ET), 117 polisitemia vera (PV), 46 primer miyelofibozis, 19 sınıflanamayan MPH] kayıtları incelenmiştir. Bulgular: İleri yaş, tanıda yüksek lökosit sayısı JAK2 mutasyon pozitifliği tromboz için risk faktörü olarak bulunmuştur. Trombosit sayımının 1000x109/L üzerinde olması kanama komplikasyonları açısından risk faktörüdür. Hidroksiüre tedavisi lösemik dönüşümle ilişkili bulunmamıştır. Bu hastalarda: Medyan takip süresi 50 ay (22,2-81,75 çeyrekler) idi. Primer miyelofibrozisli hastalar ET için 179 ay ve PV için 231 ay olan yaşam süreleri ile karşılaştırıldığında 137 ay ile en kısa yaşam süresine sahip hastalardır. Lösemik transformasyon, tromboembolik olaylar, 60 yaş üstü olmak ve anemi yaşam süresinin etkileyen faktörler olarak bulunmuştur. Sonuç: Tromboembolik komplikasyonlar MPH’da en önemli önlenebilir mortalite ve morbidite nedenidir. İlaç düzenlemeleri çoğunlukla hemoglobin ve trombosit sayımlarına göre yapılmaktadır. Bu çalışmamızdaki hasta popülasyonundan elde ettiğimiz veriler lökositoz ve JAK2 pozitifliğinin hemoglobin ve trombosit sayımlarından daha önemli risk faktorleri olduğu savını desteklemektedir.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Chronic Disease; Female; Hemorrhage; Humans; Hydroxyurea; Janus Kinase 2; Male; Middle Aged; Mutation; Myeloproliferative Disorders; Quinazolines; Retrospective Studies; Risk Factors; Turkey; Venous Thromboembolism; Young Adult

Patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) like polycythemia vera and essential thrombocythemia are at increased risk of arterial and venous thrombosis. Strategies of prevention may consist of platelet aggregation inhibitors and/or cytoreductive agents depending on the underlying disease and the individual risk. Clinical evidence for management of acute venous thromboembolic events in MPN patients is limited. Modality and duration of therapeutic anticoagulation after venous thrombosis has to be evaluated critically with special regard to the increased risk for spontaneous bleeding events associated with the underlying diseases. Both for therapy of the acute event and for secondary prophylaxis, low-molecular-weight heparins should preferentially be used. A prolongation of the therapeutic anticoagulation beyond the usual 3 to 6 months can only be recommended in high-risk settings and after careful evaluation of potential risks and benefits for the individual patient. New direct oral anticoagulants (NOAC) should not preferentially be used due to lack of clinical experience in patients with MPN and potential drug interactions (e.g. with JAK inhibitors). Consequent treatment of the underlying myeloproliferative disease and periodical evaluation of the response to therapy is crucial for optimal secondary prophylaxis of thromboembolic events in those patients.

Topics: Anticoagulants; Disease Susceptibility; Drug Interactions; Female; Hemorrhage; Heparin, Low-Molecular-Weight; Humans; Hydroxyurea; Incidence; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Male; Myeloproliferative Disorders; Phlebotomy; Platelet Aggregation Inhibitors; Postoperative Complications; Pregnancy; Pregnancy Complications, Hematologic; Preoperative Care; Protein Kinase Inhibitors; Quinazolines; Secondary Prevention; Thrombophilia; Venous Thromboembolism; von Willebrand Diseases