Loss of Asxl1 Cooperates with Oncogenic Nras to Drive CMML Progression
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Chronic myelomonocytic leukemia
Myeloproliferative neoplasm
Chronic myelomonocytic leukemia
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Myelofibrosis is a clonal myeloproliferative neoplasm associated with the proliferation of hematopoietic stem cells, increased bone marrow fibrosis, extramedullary hematopoiesis, hepatosplenomegaly, abnormal cytokine production, and constitutional symptoms. These and many other factors contribute to the development of anemia in myelofibrosis patients.
Extramedullary hematopoiesis
Ruxolitinib
Myeloproliferative neoplasm
Hepatosplenomegaly
Aplastic anemia
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SETBP1 mutations in 415 patients with primary myelofibrosis or chronic myelomonocytic leukemia: independent prognostic impact in CMML
Chronic myelomonocytic leukemia
Hematology
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Primary myelofibrosis (PMF) is a Ph-negative myeloproliferative neoplasm (MPN), that manifests bone marrow fibrosis, cytopenias, splenomegaly, and constitutional symptoms. The conventional therapeutic options for patients with PMF consist of management of anemia, use of cytoreductive and immunomogulatory agents, and splenectomy or splenic irradiation. Cure is only achievable through allogeneic haematopoietic stem cell transplantation. The discovery of crucial role of JAK2 signaling in pathogenesis of PMF has resulting in new JAK2 inhibitor therapy, such ruxolitinib or other investigated molecules.
Ruxolitinib
Myeloproliferative neoplasm
Constitutional symptoms
Extramedullary hematopoiesis
Pathogenesis
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Hybrid or Mixed Myelodysplastic/Myeloproliferative Disorders – Epidemiological Features and Overview
The WHO-category Myelodysplastic/Myeloproliferative neoplasms (MDS/MPNs) recognizes a unique group of clonal myeloid malignancies exhibiting overlapping features of myelodysplastic as well as myeloproliferative neoplasms. The group consists of chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia, BCR-ABL1 -negative (aCML), juvenile myelomonocytic leukemia (JMML), myelodysplastic/myeloproliferative neoplasm with ringed sideroblasts and thrombocytosis (MDS/MPN-RS-T), and myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U). The most frequent entity in this category is CMML, while all other diseases are extremely rare. Thus, only very limited data on the epidemiology of these subgroups exists. An appropriate diagnosis and classification can be challenging since the diagnosis is still largely based on morphologic criteria and myelodysplastic as well as myeloproliferative features can be found in various occurrences. The diseases in this category share several features that are common in this specific WHO-category, but also exhibit specific traits for each disease. This review summarizes published data on epidemiological features and offers a brief overview of the main diagnostic criteria and clinical characteristics of the five MDS/MPN subgroups.
Chronic myelomonocytic leukemia
Myeloproliferative neoplasm
Myeloproliferative Disorders
Juvenile myelomonocytic leukemia
Thrombocytosis
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Myeloproliferative neoplasm
Myeloproliferative Disorders
Neoplasm
Hematology
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Myeloproliferative neoplasm
Ruxolitinib
Myeloproliferative Disorders
Neoplasm
Critical appraisal
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Myeloproliferative neoplasm
Ruxolitinib
Thrombocytosis
Myeloproliferative Disorders
Extramedullary hematopoiesis
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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 U2AF1, were significantly more frequent in PMF than in post-PV/ET MF (33 vs. 6%; P = 0.015). There were also striking differences in clonal architecture. These data indicate different genomic spectrums between PMF and post-PV/ET MF.
Myeloproliferative neoplasm
Hematology
Myeloproliferative Disorders
Neoplasm
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Prior studies have investigated patients' characteristics, treatments, and outcomes for older adults with myelodysplastic syndromes, but most failed to distinguish chronic myelomonocytic leukemia. Recognizing potentially important differences between the diseases, we undertook a population-based comparison of baseline characteristics, treatments, and outcomes between older adults with chronic myelomonocytic leukemia and myelodysplastic syndromes. The patients' data were obtained from Surveillance Epidemiology and End Results registry data from 2001-2005, linked to Medicare claims. Baseline characteristics, treatment (red blood cell transfusions, hematopoietic growth factors, hypomethylating agents, chemotherapy or transplantation), progression to acute myeloid leukemia, and overall survival were compared using bivariate techniques. Multivariate logistic regression estimated differences in treatments received. Cox proportional hazard models estimated the effects of chronic myelomonocytic leukemia relative to myelodysplastic syndromes on progression-free survival. A larger proportion of patients with chronic myelomonocytic leukemia (n=792), compared to patients with myelodysplastic syndromes (n=7,385), failed to receive any treatment (25% versus 15%; P
Chronic myelomonocytic leukemia
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