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Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity
Myelofibrosis is a rare myeloproliferative disorder. The detailed descriptions of myelofibrosis in children and adolescents is limited to a few case series and case reports describing fewer than 100 patients, thus suggesting the extreme rarity of this ...
Fabiola Guerra+12 more
doaj +1 more source
Mutations and prognosis in primary myelofibrosis [PDF]
Patient outcome in primary myelofibrosis (PMF) is significantly influenced by karyotype. We studied 879 PMF patients to determine the individual and combinatorial prognostic relevance of somatic mutations. Analysis was performed in 483 European patients and the seminal observations were validated in 396 Mayo Clinic patients.
VANNUCCHI, ALESSANDRO MARIA+31 more
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Myelofibrosis and Pancytopenia Associated With Primary Hyperparathyroidism [PDF]
OBJECTIVE: Primary hyperparathyroidism (PHPT) has varied clinical presentations. Hematologic abnormalities secondary to PHPT have been described before. However, pancytopenia as the initial presentation has rarely been reported. We report a patient with PHPT who presented for evaluation of pancytopenia.
Rajan, R+8 more
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Primary myelofibrosis shows histological and pathogenetic overlap with essential thrombocythemia and polycythemia vera. Several diagnostic classifications have been proposed for primary myelofibrosis, although little is known about their clinical utility.
Philip A. Beer+2 more
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Primary myelofibrosis is characterized by clonal myeloproliferation, dysmegakaryopoiesis, extramedullary hematopoiesis associated with myelofibrosis and altered stroma in the bone marrow and spleen.
Christophe Desterke+16 more
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BACKGROUND: The detection of molecular and cytogenetic alterations is important for the diagnosis, prognosis and classification of myeloproliferative neoplasms. OBJECTIVE: The aim of this study was to detect the following mutations: JAK2 V617F, JAK2 exon
Leonardo Caires dos Santos+5 more
doaj +1 more source
Background The World Health Organization classification of myeloproliferative neoplasms discriminates between essential thrombocythemia and the prefibrotic phase of primary myelofibrosis.
Thomas Buhr+5 more
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Refractory gout in primary myelofibrosis [PDF]
![Figure][1] A 66-year-old man from the Dominican Republic presented to the Myeloproliferative Neoplasms Clinic at Mount Sinai with a 10-year history of untreated JAK2V617F- positive primary myelofibrosis (PMF).
Sangeetha Venugopal, John Mascarenhas
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Primary Myelofibrosis with Mesenteric Extramedullary Haematopoiesis [PDF]
Primary myelofibrosis or chronic idiopathic myelofibrosis is a myeloproliferative neoplasm characterized by proliferation of abnormal haematopoietic stem cells in bone marrow resulting in replacement of marrow with fibrotic scar tissue.
Saurabh Kumar+4 more
doaj +1 more source
In primary myelofibrosis, extra-domain A fibronectin (EDA-FN), the result of alternative splicing of FN gene, sustains megakaryocyte proliferation and confers a pro-inflammatory phenotype to bone marrow cell niches. In this work we assessed the levels of
Alessandro Malara+6 more
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