Background: Essential thrombocythemia (ET) is a rare disease in which there an increase the platelet count of more than 450,000/mm3. An number platelets occurs due to increased proliferation megakaryocyte series. Although ET, it has impaired function. ET clinical manifestations 50% cases are asymptomatic, but symptomatic can be vascular occlusive events or microvascular thrombosis.
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SUMMARY OBJECTIVE: The aim of this study was to compare the incidence factors associated with an increased risk thrombosis in patients essential thrombocythemia. METHODS: A total 200 followed-up our unit a diagnosis thrombocythemia 13 years were analyzed retrospectively. RESULTS: Of participants, 60.5% females and 39.5% males, overall mean (±SD) age 54.93 (±14.21) years. In 119 patients, Janus ...

Hereditary thrombocythemia is a rare autosomal dominant disorder caused by mutations in either the thrombopoietin gene (TPO) or its receptor c-MPL. TPO mutations described so far lead to thrombopoietin overproduction through increased translation of m-RNA. Unilateral transverse reduction limb defects are usually sporadic and generally thought to be caused by vascular disruptions. Reports of inh...

The discovery of the JAK2V617F mutation triggered an unexpected flowering of basic and clinical studies in the field of myeloproliferative neoplasms (MPNs), resulting after just a few years in an exceptional amount of new information. One important consequence of those new findings was the modification of the World Health Organization classification and diagnostic algorithms for these diseases,...

1216. 3. Marasca R, Zucchini P, Galimberti S, et al. Missense mutation in the PML/ RARa ligand binding domain in ATRA-resistant As2O3 sensitive relapsed acute promyelocytic leukemia. Haematologica. 1999;84:963-968. 4. Hughes TP, Morgan HGJ, Martiat P, Goldman JM. Detection of residual leukemia after bone marrow transplant for chronic myeloid leukemia: role of polymerase chain reaction in predic...

Plasma von Willebrand factor (vWF) in eleven patients with essential thrombocythemia was studied quantitatively and qualitatively to assess its role in the development of bleeding tendency. Five patients with prolonged bleed ing time including three with clinical bleeding tendencies showed decreased ristocetin cofactor (VIIIR: RCo) and factor VIII-related antigen (VIIIR: Ag). On the other hand,...

The development of acute lymphoblastic leukemia in an existing myeloproliferative neoplasm is rare with historical cases unable to differentiate between concomitant malignancies or leukemic transformation. Molecular studies of coexisting JAK2 V617F-positive myeloproliferative neoplasms and mature B cell malignancies indicate distinct disease entities arising in myeloid and lymphoid committed he...

Moyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic angiographic appearance. Although the pathophysiology is unclear, hematologic disorders have been...

Our understanding of the genetic basis of myeloproliferative neoplasms began in 2005, when the JAK2 (V617F) mutation was identified in polycythemia vera, essential thrombocythemia, and primary myelofibrosis. JAK2 exon 12 and MPL exon 10 mutations were then detected in subsets of patients, and subclonal driver mutations in other genes were found to be associated with disease progression. Recentl...

Essential thrombocythemia (ET) is a clonal disorder of myeloid stem cells that causes thrombocytosis. As a result, ET can lead to vascular thrombosis and tissue ischemia; the association of coronary artery abnormalities such as myocardial infarction or unstable angina is rare. Here we describe a 45,-year-old male patient with essential thrombocythemia who presented with unstable angina. Electiv...