Abstract
Myelodysplastic syndromes (MDS) are defined in the World Health Organization classification of tumors of hematopoietic and lymphoid tissues as a group of clonal hematopoietic cell diseases characterized by cytopenia, dysplasia in one or more of myeloid cell lines, ineffective hematopoiesis, and an increased risk of progression to acute myeloid leukemia (AML). To determine the incidence of MDS in the pediatric hematology and oncology department of hospital 20 August 1953. Descriptive retrospective study made at the Pediatric Hematology and Oncology Department of hospital 20 August 1953 of the CHU Ibn Rochd Casablanca, including all patients with MDS confirmed on myelogram or BOM, diagnosed between January 2014 and December 2018. Patients with CMML, MDS/MPS border syndromes, or AML were excluded from our study. We found 272 patients diagnosed with MDS over the 15-year period from January 2004 to December 2018. The median incidence of MDS in our study was 19 cases/year, the median age was 59.5 years. According to the age groups, we found that 108 patients (or 40%) represented the age group of young adults (31-60 years), 67 patients (or 25%) represented the age group of elderly subjects (61-74 years), 60 patients (20%) represented the age group of very old subjects (over 75 years). The sex ratio (M/F) is 0.74. We noted that 1% of patients were genetically exposed and 16% of patients were exposed to various risk factors. The history of autoimmune diseases was found in 18% of patients in our series. 91% of patients had clinical signs. Therapeutically, we found that 74% of patients received symptomatic treatment. Fifteen patients in our series received specific treatment. Median survival is 5.63 months Conclusions: Myelodysplastic syndromes present a heterogeneous group of clonal hemopathies characterized by peripheral cytopenias and the risk of progression to acute leukemia. Diagnosis is currently based on bone marrow cytology, but the contribution of cytogenetic and molecular biology seems to go beyond prognostic interest (early diagnosis, prediction of therapeutic response). While the treatment of low-risk myelodysplastic syndromes is based on the correction of cytopenias, that of high-risk myelodysplastic syndromes aims to control the leukemic clone.
Published Version
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