20 April 2004
Myelodysplastic Syndromes (MDS) with various clinical and cytogenetic presentation
Grażyna Sobol, Jadwiga Małdyk, Agnieszka Mizia, Halina WośCase Rep Clin Pract Rev 2004; 5(null):5-8 :: ID: 12280
Abstract
Background: Myelodysplastic Syndromes (MDS) are heterogeneous groups of clonal stem cell disorders.
Ineffective hematopoiesis manifests itself as dyserythropoiesis, dysgranulopoiesis, and
dysmegakaryopoiesis, which are elements of a typical clinical presentation.
Case Report: We present 2 different cases of this rare syndrome with characteristic cytological, histopatological and cytogenetic abnormalities. The optimum treatment for childhood MDS has not been yet defined, but the allogenic bome marrow transplantation (allo-BMT) seems to be the method of choice. Our patients have undergone the stem cell transplantation.
Conclusions: In children with peripheral blood morphology abnormalities such as cytopenias or hyperleukocytosis with the presence of young form in peripheral blood smear in diagnostic procedure myelodysplastic syndrome should be considered. Cytogenetic examination should be conducted in children with myelodysplastic syndromes for confirmation of diagnosis and for prognosis. Patients with final diagnosis of MDS ought to be referred for emergency preparation to allotransplant of bone marrow (donor search), which improves the chances of remission.
Keywords: Myelodysplastic syndromes, Bone Marrow Transplantation, children
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