Abstract
Aim: Myelodysplastic syndromes are a group of haematologic disorders characterized by varying degrees of cytopenias and a propensity to leukaemic transformation. The aim of this study is to determine the prevalence, clinical and laboratory characteristics and the outcome of management in Nigerians with this disorder. Methods: Ten patients who have full clinical and laboratory information were retrospectively studied. Data extracted included demographic parameters, clinical features at presentation, haematological parameters, including bone marrow cytology (and cytogenetic findings, where available), management instituted and outcome of such therapy. Results: Ten patients with de novo MDS were managed and followed-up for a median period of 3 months. The majority (90.0%) were aged 50 years or above with a median age of 65 years. All presented with symptoms of cytopenias such as anaemia (100%), neutropenia (50.0%), and thrombocytopenia (10.0%). Patients had mainly supportive management such as blood product supports. One patient, however, in addition received growth factor and cytotoxic chemotherapy, while one received cytotoxic drugs alone. These were however not adequate due to financial constraints. Eight deaths were recorded (88.9%), either cytopenia related (five) or renal failure (three). Cause of death in one was not known as he died at home. The mean survival was 50.5 ± 96.1 weeks (range=2.1–308.4 weeks) Conclusion: It could be concluded that though clinical and laboratory features of Nigerians with MDS are similar to what is obtained from other parts of the world, non-availability of both specific and supportive drugs, and poor socio-economic status of most patients contributed significantly to the poor outcome recorded in this report.
Highlights
The myelodysplastic syndromes (MDS)are a group of acquired invariably fatal clonal haematopoietic stem cells disorders characterised by ineffective haematopoiesis, peripheral cytopenias [1] and potential to evolve into acute leukaemia
All presented with symptoms of cytopenias such as anaemia (100%), neutropenia (50.0%), and thrombocytopenia (10.0%)
It could be concluded that though clinical and laboratory features of Nigerians with MDS are similar to what is obtained from other parts of the world, non-availability of both specific and supportive drugs, and poor socio-economic status of most patients contributed significantly to the poor outcome recorded in this report
Summary
Are a group of acquired invariably fatal clonal haematopoietic stem cells disorders characterised by ineffective haematopoiesis, peripheral cytopenias [1] and potential to evolve into acute leukaemia. The major clinical problems manifested by these patients are related to the cytopenias, most especially anaemia, most often transfusion-dependent. Based on percentage marrow blasts and ringed sideroblasts, Bennet, et al [4] classified MDS into 5 morphologic subgroups, the relatively indolent refractory anaemia (RA, blasts < 5%) and refractory anaemia with ringed sideroblasts (RARS, blasts < 5%), the aggressive refractory anaemia with excess blasts (RAEB, blasts 5-20%)) and the more highly aggressive refractory anaemia with excess blasts in transition (RAEB-T, blasts 20-30%), as well as the incurable chronic Patients Mean age (yr) Mean PCV (%).
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