Chronic myelomonocytic leukemia – a review of present diagnostic and treatment options

Czech version

Authors: P. Rohoň; M. Jarošová; K. Indrák
Authors‘ workplace: Hemato-onkologická klinika FN a LF UP v Olomouci
Published in: Transfuze Hematol. dnes,20, 2014, No. 2, p. 58-66.
Category: Comprehensive Reports, Original Papers, Case Reports

Overview

Chronic myelomonocytic leukemia (CMML) is a clonal disorder of hematopoietic stem cell characterized by features of a myelodysplastic syndrome (MDS) and a myeloproliferative neoplasm (MPN). It occurs mainly in older people, mostly in men, and often transforms into acute myeloid leukemia (AML). The most common symptoms of CMML reflect hypercatabolism, the present bicytopenia, (hepato)splenomegaly and organ infiltration with monocytes. It is characterized by absolute monocytosis (> 1 x 10⁹/l) in the peripheral blood that persists longer than 3 months. Cytogenetic examination confirmed the presence of chromosomal aberrations in approximately 40% of cases, but they are not specific for this disease. Nowadays, significant attention is focused on the TET2 gene mutation – its prognostic significance is not fully understood. JAK2 (V617F) mutation is present mainly in myeloproliferative variant of MP-CMML. The disease has variable clinical course and a very poor prognosis. Although new information from molecular biology of CMML are implemented to the treatment strategy, the results are still unsatisfactory. The objective of this review is to summarize recent information about the disease pathogenesis and the proposal for a treatment algorithm.

Key words:
chronic myelomonocytic leukemia, diagnostics, pathogenesis, TET2 gene, therapy

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