Hemorrhage in Essential Thrombocythemia or Polycythemia Vera: Epidemiology, Location, Risk Factors, and Lessons Learned from the Literature

Abstract

Hemorrhage is a well-known complication of essential thrombocythemia (ET) and polycythemia vera (PV), but evidence-based data on its management and prevention are lacking to help inform clinicians. In this review, appropriate published data from the past 15 years regarding bleeding epidemiology, classification, location, and risk factors are presented and discussed. Research was conducted using the Medline database. The bleeding classifications were heterogeneous among the collected studies. The median incidences of bleeding and major bleeding were 4.6 and 0.79% patients/year, in ET patients and 6.5 and 1.05% patients/year in PV patients, respectively. The most frequent location was the gastrointestinal tract. Bleeding accounted for up to 13.7% of deaths, and cerebral bleeding was the main cause of lethal hemorrhage. Thirty-nine potential risk factors were analyzed at least once, but the results were discrepant. Among them, age >60 years, bleeding history, splenomegaly, myeloproliferative neoplasm subtype, and platelet count should deserve more attention in future studies. Among the treatments, aspirin seemed to be problematic for young patients with ET (especially CALR-mutated ET patients) and anagrelide was also identified as a bleeding inducer, especially when associated with aspirin. Future studies should analyze bleeding risk factors in more homogeneous populations and with common bleeding classifications. More tools are needed to help clinicians manage the increased risk of potentially lethal bleeding events in these diseases.

PEAR1 Genetic Variants in Essential Thrombocythemia: The Prevalence and Association with Hematological Parameters and ET Mutations

“…Another hematological symptom in ET patients is hemorrhage, which occurs due to platelet dysfunction in the coagulation system despite the high platelet counts. Studies have revealed that in ET patients, hemorrhage is less frequent than thrombosis (3 to 18% of patients) [23]. In the present study, the hemorrhage incidence rate was higher in women than men (f:m 2.0).…”

Bleeding complications in bcr‐abl‐negative myeloproliferative neoplasms (MPN): A retrospective single‐center study of 829 MPN patients

“…Nicol et al 7 identified 39 potential risk factors for bleeding events in their extensive literature review, which included over 15 000 patients with essential thrombocythemia (ET) and polycythemia vera (PV). Remarkably, only 37 fatal hemorrhages were recorded.…”

“…Of note, bleeding occurred significantly more often in MF patients, but bleeding under anticoagulants other than DOACs or under antiplatelet agents was not studied. Recently, Nicol et al 7 reported an incidence for all bleeding events ranging from 0.1 to 6.6% and for major bleeding from 0.3 to 5.3% per patient/year in a comprehensive literature review that included 38 studies involving over 10 000 ET and over 5000 PV patients. A total of 39 different potential risk factors for hemorrhage were identified (including age, gender, MPN diagnosis, mutation status and anticoagulation therapies), but no data were presented for MF patients.…”

PEAR1 Genetic Variants in Essential Thrombocythemia: A New Study of The Prevalence and Association of PEAR1 Variants with Hematological Parameters and ET Mutations

“…Although this neoplasm is associated with longer overall survival, patients' life expectancy may be reduced due to the occurrence of thrombosis, hemorrhage, and the risk of hematological malignancies transformation (such as secondary myelo brosis or acute myeloid leukemia) compared to the general population. (23,26). The overall risk of arterial or venous thrombosis in these patients is estimated at 1-3% per patient-year; Therefore, thrombosis is a lifethreatening risk factor in ET patients (27).…”