Shedding new light on the magnitude of thrombosis risk in patients with myeloproliferative neoplasms
Menée en Suède à partir de données portant sur 9 429 patients atteints d'un syndrome myéloprolifératif et sur 35 820 témoins entre 1987 et 2009, cette étude de cohorte analyse les facteurs associés au risque de thrombose artérielle et veineuse
Myeloproliferative diseases were first described by William Dameshek in 1951. In 2008, the World Health Organization established a new classification system and introduced the term “myeloproliferative neoplasms” (MPNs). Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are the most prevalent MPNs and are characterized by overproduction of leukocytes, erythrocytes, or platelets; development of bone marrow fibrosis; leukemic transformation; and arterial and venous thrombosis. When Dameshek proposed the term “myeloproliferative diseases,” he also proposed the presence of a then-undiscovered stimulus that drove proliferation. We now understand that mutation of the JAK2 gene, JAK2 V617F, is the most common stimulus, occurring in 95% of patients with PV and 60% of those with ET or PMF. Myeloproliferative neoplasms are relatively rare; are acquired in middle to older age; and are, despite their classification as neoplasms, indolent diseases, with survival measured in decades (1). Yet, MPNs exhibit marked variability in disease presentation and outcomes, especially with regard to thrombotic events.
Annals of Internal Medicine , éditorial, 2017