Molecular genetics of chronic neutrophilic leukemia, chronic myelomonocytic leukemia and atypical chronic myeloid leukemia
نویسندگان
چکیده
According to the 2008 World Health Organization classification, chronic neutrophilic leukemia, chronic myelomonocytic leukemia and atypical chronic myeloid leukemia are rare diseases. The remarkable progress in our understanding of the molecular genetics of myeloproliferative neoplasms and myelodysplastic/myeloproliferative neoplasms has made it clear that there are some specific genetic abnormalities in these 3 rare diseases. At the same time, there is considerable overlap among these disorders at the molecular level. The various combinations of genetic abnormalities indicate a multi-step pathogenesis, which likely contributes to the marked clinical heterogeneity of these disorders. This review focuses on the current knowledge and challenges related to the molecular pathogenesis of chronic neutrophilic leukemia, chronic myelomonocytic leukemia and atypical chronic myeloid leukemia and relationships between molecular findings, clinical features and prognosis.
منابع مشابه
A Rare Case of Acute Myeloid Leukemia with Translocation (3:3) Presenting with Features of Chronic Myelomonocytic Leukemia
Background: Acute Myeloid Leukemia (AML) with translocation (3,3) is a form of AML that may present de novo or may arise from a previous myelodysplastic syndrome. It is often associated with normal or elevated peripheral blood platelet count and increased bone marrow megakaryocytes with associated multi lineage dysplasia. A subset of patients present with hepatosplenomegaly while a few cases h...
متن کاملSignaling pathways involved in chronic myeloid leukemia pathogenesis: the importance of targeting Musashi2-Numb signaling to eradicate leukemia stem cells
Objective(s): Chronic myeloid leukemia (CML) is a myeloid clonal proliferation disease defining by the presence of the Philadelphia chromosome that shows the movement of BCR-ABL1. In this study, the critical role of the Musashi2-Numb axis in determining cell fate and relationship of the axis to important signaling pathways such as Hedgehog and Notch that are essential ...
متن کاملAtypical myeloproliferative disorders: diagnosis and management.
Myeloid disorders constitute a subgroup of hematological malignancies that is separate from lymphoid disorders. The World Health Organization system for classification of tumors of the hematopoietic system divides myeloid disorders into acute myeloid leukemia and chronic myeloid disorders based on the presence or absence, respectively, of acute myeloid leukemia--defining morphological and cytog...
متن کاملFrequency of BCR-ABL Fusion Transcripts in Iranian Azeri Turkish patients with Chronic Myeloid Leukemia
Background: The Philadelphia chromosome (Ph) characterized by t (9; 22) (q34; q11.2) is a reciprocal translocation giving rise to a chimeric BCR-ABL fusion gene. Incidence of Ph chromosome is over 98% in Patients with Chronic Myeloid Leukemia (CML) and around 20% in acute lymphoblastic leukemia (ALL). The finding of this fusion gene is essential for diagnosis of CML by detection of various fusi...
متن کاملMolecular Pathogenesis of Chronic MPDs
Chronic myeloproliferative disorders (MPDs), first proposed by Dameshek in 1951, are clonal hematopoietic stem cell disorders characterized by proliferation of 1 or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature cells in peripheral blood.1 According to the World Health Organization classification, MPDs include polycythemia vera (PV), essential thrombo...
متن کامل