PedAM
Pediatric Disease Annotations & Medicines
| Disease | primary myelofibrosis |
| Phenotype | C0001815|myelofibrosis |
| Sentences | 15 |
| PubMedID- 20359349 | Clonal dysregulation linked to myeloproliferative neoplasms (mpn) arising from acquired mutations in the haematopoietic progenitor cells lead to a wide range of clonal haematological malignant diseases including polycythemia vera (pv), essential thrombocythemia (et), myeloid metaplasia with myelofibrosis (mmm), chronic myelomonocytic leukemia (cmml), chronic myelogenous leukemia (cml), hypereosinophilic syndrome (hes), and systemic mast cell disease (smcd)[1]. |
| 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- 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- 23259436 | Altogether, the stepwise progress of myelofibrosis in cimf was associated with expression of a defined subset of target genes as shown in sequential trephine biopsies of cimf patients. |
| PubMedID- 22548075 | Most of the reported pulmonary extramedullary hematopoiesis is seen in myelofibrosis with myeloid metaplasia, a subgroup of chronic myeloproliferative disorders. |
| PubMedID- 23208557 | This mutation has been identified in most polycythemia vera (pv) patients, approximately half of essential thrombocythemia (et) and chronic idiopathic myelofibrosis with extramedullary hematopoiesis (cimf) patients, and in a substantial population of other chronic myeloproliferative disease (cmpd) patients [40]. |
| PubMedID- 21870730 | [myeloid metaplasia with myelofibrosis associated with acute coronary syndrome. |
| PubMedID- 24744667 | Also suggested that pv patients homozygous for jak2v617f are more prone to transformation into myelofibrosis with myeloid metaplasia. |
| PubMedID- 19785025 | Chronic idiopathic myelofibrosis (cim) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra-medullary hematopoesis (emh), and varying degree of myelofibrosis. |
| 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- 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. |
| PubMedID- 22704352 | Report reduction of myelofibrosis in mice with jak2+ primary myelofibrosis (pmf) when pmf cells are treated sequentially with decitabine and saha (vorinostat) or trichostatin a in vitro prior to transplantation (wang et al. |
| PubMedID- 21224796 | The role of splenectomy in myelofibrosis with myeloid metaplasia. |
| PubMedID- 20615740 | Objective: lenalidomide (ldm) is an immunomodulatory and anti-angiogenic drug which has been shown to be effective in several haematological disorders (multiple myeloma [mm], myeloid metaplasia with myelofibrosis [mf] and myelodysplastic syndrome [mds]). |
| PubMedID- 24880208 | Essential thrombocytosis (et) falls under the umbrella of myeloproliferative disorders, which also includes chronic myelogenous leukemia, polycythemia vera, and myelofibrosis with myeloid metaplasia. |
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