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PedAM

Pediatric Disease Annotations & Medicines




Disease primary myelofibrosis
Phenotype C0001815|myelofibrosis with myeloid metaplasia
Sentences 7
PubMedID- 22958626 'idiopathic myelofibrosis' (im), 'agnogenic myeloid metaplasia' or 'myelofibrosis with myeloid metaplasia' are synonymous for a rare chronic myeloproliferative disease.
PubMedID- 22548075 Most of the reported pulmonary extramedullary hematopoiesis is seen in myelofibrosis with myeloid metaplasia, a subgroup of chronic myeloproliferative disorders.
PubMedID- 23756559 An increase in circulating cd34+ cells has been described in myelofibrosis with myeloid metaplasia in adults (30) and in raeb in adults (31) and in children, associated with a low apoptotic rate (25).
PubMedID- 23457485 Exclusion criteria were prior chronic renal dysfunction, previously treated malignancy (>4 weeks after the first chemotherapy dose), relapsing or refractory hematological malignancy, bone marrow or stem cell transplantation, low-grade or chronic malignancy (chronic myelogenous leukemia, chronic lymphocytic leukemia, low-grade nhl, multiple myeloma, myelodysplastic syndrome, myelofibrosis with myeloid metaplasia, or aplastic anemia), and absence of potentially lifespan-extending treatment options.
PubMedID- 23259545 myelofibrosis with myeloid metaplasia (mmm) is a rare malignant hematological disease of the hematopoietic stem cells with a median age at diagnosis of 60 years.
PubMedID- 24744667 Also suggested that pv patients homozygous for jak2v617f are more prone to transformation into myelofibrosis with myeloid metaplasia.
PubMedID- 22282696 Increase in fibrosis is due to liberation of cytokines such as tgf-β and pdgf from dysplastic megakaryocytes.15 the differential diagnosis includes myelofibrosis with myeloid metaplasia, chronic myelocytic leukemia and acute megakaryocytic leukemia.

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