Splanchnic Vein Thrombosis Associated with Myeloproliferative Neoplasms: A Study of the AGIMM & IWG-MRT Groups in 519 Subjects
نویسندگان
چکیده
منابع مشابه
JAK2 46/1 haplotype predisposes to splanchnic vein thrombosis-associated BCR-ABL negative classic myeloproliferative neoplasms.
The germline constitutive JAK2 haplotype, called GGCC or 46/1, s a susceptibility factor for BCR-ABL negative classic myeloproiferative neoplasms (MPNs), [1]. The mechanisms linking the aplotype block and the acquired MPN remain unclear. Two ypotheses have been proposed: the “hypermutability” of the chroosome region that could facilitate the arising of typical MPN omatic mutations [2], or the “...
متن کاملSplanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients
We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected ...
متن کاملSplanchnic vein thrombosis, the onset manifestation in JAK positive Chronic Myeloproliferative Disorders Neoplasms
BACKGROUND Patients with Myeloproliferative Neoplasms-(MPN) have a high risk of thrombotic complications. Portal vein thrombosis is a severe complication, which in many cases, appears at the onset of the disease; the risk factors are related to the presence of qualitatively altered thrombocytes and leucocytes, leading to their activation and appearance of leukocytes-platelet-aggregates; anomali...
متن کاملSplanchnic vein thrombosis in myeloproliferative neoplasms: pathophysiology and molecular mechanisms of disease.
Myeloproliferative neoplasms (MPNs) are the most common underlying prothrombotic disorder found in patients with splanchnic vein thrombosis (SVT). Clinical risk factors for MPN-associated SVTs include younger age, female sex, concomitant hypercoagulable disorders, and the JAK2 V617F mutation. These risk factors are distinct from those associated with arterial or deep venous thrombosis (DVT) in ...
متن کاملSplanchnic vein thrombosis and myeloproliferative neoplasms: molecular-driven diagnosis and long-term treatment.
Splanchnic vein thrombosis (SVT) encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis. Philadelphia-negative myeloproliferative neoplasms (MPNS) are the leading systemic cause of non-cirrhotic and non-malignant SVT and are diagnosed in 40% of BCS patients and one-third of EHPVO patients. In SVT patients the molecular marker JAK2 V6...
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ژورنال
عنوان ژورنال: Blood
سال: 2014
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v124.21.3163.3163