Progression of romiplostim myelofibrosis to myeloproliferative neoplasm

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Romiplostim-induced myelofibrosis.

A 55-year-old woman with chronic refractory idiopathic thrombocytopenic purpura (ITP) presented with gum bleeding for 1 day. Over the course of several years, she had failed, or became resistant to, multiple treatments including glucocorticoids, intravenous immunoglobulin, splenectomy, rituximab, and, recently, a 9-month course of weekly romiplostim with moderate compliance. A complete blood co...

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Non-driver mutations in myeloproliferative neoplasm-associated myelofibrosis

We studied non-driver mutations in 62 subjects with myeloproliferative neoplasm (MPN)-associated myelofibrosis upon diagnosis, including 45 subjects with primary myelofibrosis (PMF) and 17 with post-polycythemia vera or post-essential thrombocythemia myelofibrosis (post-PV/ET MF). Fifty-eight subjects had ≥1 non-driver mutation upon diagnosis. Mutations in mRNA splicing genes, especially in U2A...

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Ptch2 loss drives myeloproliferation and myeloproliferative neoplasm progression

JAK2V617F(+) myeloproliferative neoplasms (MPNs) frequently progress into leukemias, but the factors driving this process are not understood. Here, we find excess Hedgehog (HH) ligand secretion and loss of PTCH2 in myeloproliferative disease, which drives canonical and noncanonical HH-signaling. Interestingly, Ptch2(-/-) mice mimic dual pathway activation and develop a MPN-phenotype with leukoc...

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Critical appraisal of the role of ruxolitinib in myeloproliferative neoplasm-associated myelofibrosis

The recent approval of molecular-targeted therapies for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF) has dramatically changed its therapeutic landscape. Ruxolitinib, a JAK1/JAK2 tyrosine kinase inhibitor, is now widely used for first- and second-line therapy in persons with MPN-MF, especially those with disease-related splenomegaly, intermediate- or high-risk disease, and const...

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Progression of primary myelofibrosis to polycythemia vera

RATIONALE This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms. PATIENT CONCERNS The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months. DIAGNOSIS A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JA...

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ژورنال

عنوان ژورنال: Blood

سال: 2014

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood-2013-09-528778