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  • mutation
  • Although CMML does have a distinct 'genetic fingerprint' (ie, a unique frequency and pattern of mutated genes), there is no single disease-defining mutation or chromosomal abnormality. (uptodate.com)
  • characteristic
  • Similar to the closely related pediatric disease juvenile myelomonocytic leukemia (JMML), a large fraction of CMML cases display hypersensitivity to granulocyte macrophage colony-stimulating factor (GM-CSF) in vitro, which likely contributes to the monocytosis characteristic of the disease [ 11-13 ]. (uptodate.com)
  • PDGFR
  • From the involvement of PDGF in the fibrotic process, it has been suggested that the PDGF-PDGFR system might be a promising target for treating fibrotic disease ( 15 ). (rupress.org)
  • Additional genetic abnormalities have been identified in patients with SM-AHNMD ( 13 , 14 ) in whom an associated CEL may be linked to the FIP1L1-platelet-derived growth factor receptor α (PDGFRα) fusion gene ( 14 - 17 ). (aacrjournals.org)
  • gene
  • An important gene associated with 8p11 Myeloproliferative Syndrome is FGFR1 (Fibroblast Growth Factor Receptor 1), and among its related pathways/superpathways are HIV Life Cycle and CREB Pathway . (malacards.org)
  • The tyrosine kinase (TK) inhibitor dasatinib is 325-fold more potent against Bcr-Abl TK than imatinib in vitro , significantly inhibiting wild-type KIT and platelet-derived growth factor receptor β TKs, and is active against cells carrying the mutant KIT-D816V gene. (aacrjournals.org)
  • The protein encoded by this gene is a cell surface tyrosine kinase receptor for members of the platelet-derived growth factor family. (nih.gov)
  • The Fip1-like1 (FIP1L1)-platelet-derived development aspect receptor alpha blend gene (F/P) arising in the pluripotent hematopoietic control cell (HSC),causes 14% to 60% of sufferers with hypereosinophilia symptoms (HES). (aksumuniversity.org)
  • Launch An interstitial removal on chromosome 4q12 outcomes in the development of the Fip1-like1 (FIP1M1)-platelet-derived development aspect receptor leader blend gene (Y/G), which leads to the incidence of chronic eosinophilic leukemia (CEL) . (aksumuniversity.org)
  • Studies in Gata1 - knockout mice , i.e. mice lacking the Gata1 gene, indicate that this gene is essential for the development and maintenance of blood-based and/or tissue-based hematological cells, particularly red blood cells and platelets but also eosinophils , basophils , mast cells , and dendritic cells . (wikipedia.org)
  • Tissue factor (TF) can also stimulate the transcription of the gene encoding VEGF-A. The relationship between coagulation and angiogenesis in myeloproliferative neoplasms is not fully understood. (mdpi.com)
  • hematologic
  • Chronic phase: 400 mg once daily; may be increased to 600 mg daily, if tolerated, for disease progression, lack of hematologic response after 3 months, lack of cytogenetic response after 6-12 months, or loss of previous hematologic or cytogenetic response. (scribd.com)
  • MPDs
  • Considering that the detection of the V617F JAK2 mutation is likely to have a major impact on the way patients with myeloproliferative diseases (MPDs) are diagnosed, we have investigated if its presence could be revealed by the immunohistologic detection of phosphorylated STATs. (bloodjournal.org)
  • fibrotic
  • Evidence for the importance of platelet-derived growth factor (PDGF) signaling in the fibrotic process is provided by reports showing that a number of fibrogenic mediators such as TGF-β, IL-1, TNF-α, bFGF, and thrombin exhibit PDGF-dependent profibrotic activities ( 6 , 11 - 14 ). (rupress.org)
  • CMPD
  • Impetus for the clinical investigation of novel targeted agents for the treatment of CMPD has been provided by advances in molecular characterization of these diverse diseases. (aacrjournals.org)
  • tumors
  • This review summarizes the physiologic activity of PDGF, the expression of PDGF during embryonal development, and the roles of PDGF expression in nonmalignant disease and in different tumors. (nih.gov)
  • molecular
  • Indeed, hematology has been at the heart of such fundamental work as the understanding of iron regulation in red blood cell mediated tissue oxygenation, 1 or the deciphering of the intricate molecular interactions between endothelial cells, platelets and plasmatic proteins in the early stages of hemostasis. (haematologica.org)
  • stem cell
  • Pediatric patients with Ph+ CML in chronic phase who are newly diagnosed or whose disease has recurred after stem cell transplant or who are resistant to interferon-alpha therapy. (rxdrugsinfo.com)
  • patients
  • Although these agents have not been successful in curing CML, TKIs have been shown to achieve long-term disease control in a majority of treated patients and have become the initial treatment of choice. (oncolink.org)
  • Treatment options for patients with CML are quite complex and depend on the phase of disease, availability of a bone marrow donor and patient choice. (oncolink.org)
  • The clinical course, prognosis, and response to certain drugs vary greatly among patients and among disease variants. (waojournal.org)
  • Living with a genetic or rare disease can impact the daily lives of patients and families. (nih.gov)
  • In patients with cardiovascular disease. (togetherrxacces.com)