Chronic Myeloproliferative Disorders
AbstractThe Philadelphia chromosome-negative chronic myeloproliferative disorders (CMPD), polycythemia vera (PV), essential thrombocythemia (ET) and chronic idiopathic myelofibrosis (IMF), have overlapping clinical features but exhibit different natural histories and different therapeutic requirements.
Gianni Tognoni+6 more
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Abstract In 1951 William Dameshek classified polycythemia vera (PV), essential thombocytosis (ET), and primary myelofibrosis (PMF) as pathogenetically related myeloproliferative disorders (MPD). Subsequent studies demonstrated that PV, ET, and PMF are clonal disorders of multipotent hematopoietic progenitors.
Ross L, Levine, D Gary, Gilliland
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The Thrombopoietin Receptor, MPL, Is a Therapeutic Target of Opportunity in the MPN
The myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis and primary myelofibrosis share driver mutations that either activate the thrombopoietin receptor, MPL, or indirectly activate it through mutations in the gene for JAK2, its ...
Jerry L. Spivak, Alison R. Moliterno
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Systemic mastocytosis with associated myeloproliferative disease and precursor B lymphoblastic leukaemia with t(13;13)(q12;q22) involving FLT3. [PDF]
Systemic mastocytoses represent neoplastic proliferations of mast cells. In about 20% of cases systemic mastocytoses are accompanied by clonal haematopoietic non-mast cell-lineage disorders, most commonly myeloid neoplasms.
Dirnhofer, S.+7 more
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The JAK2 gene encodes for a non‐receptor tyrosine kinase that plays a key role in the JAK/STAT signaling transfer pathway. Genetic polymorphisms of this gene have been indicated to be associated with myeloproliferative neoplasm‐associated thrombosis in ...
Nguyen Thy Ngoc+3 more
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Gaucher Disease and Myelofibrosis: A Combined Disease or a Misdiagnosis? [PDF]
Background: Gaucher disease (GD) and primary myelofibrosis (PMF) share similar clinical and laboratory features, such as cytopenia, hepatosplenomegaly, and marrow fibrosis, often resulting in a misdiagnosis.
Cardarelli, L+5 more
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The role of the JAK2 GGCC haplotype and the TET2 gene in familial myeloproliferative neoplasms
Background Myeloproliferative neoplasms constitute a group of diverse chronic myeloid malignancies that share pathogenic features such as acquired mutations in the JAK2, TET2, CBL and MPL genes.
Damla Olcaydu+10 more
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Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis [PDF]
Background. The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients. Aim.
Aprile, Francesca+7 more
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Chronic myeloproliferative disorders: A rarest case with oral manifestations and dental management
Chronic myeloproliferative disorders (CMPD) are rarest hematological disorders (malignant myeloid neoplasms). The three most common chronic myeloproliferative disorders are polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis.
Pritesh B Ruparelia+3 more
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Is porto sinusoidal vascular disease to be actively searched in patients with portal vein thrombosis? [PDF]
Porto sinusoidal vascular liver disease (PSVD) and portal vein thrombosis (PVT) are distinct vascular liver diseases characterized, respectively, by an intrahepatic and a prehepatic obstacle to the flow in the liver portal system. PVT may also occur as a
D'Amati, G.+4 more
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