Myelodysplastic syndrome(MDS) is a group of acquired conditions characterized by progressive bone marrow failure associated with normocellular or hypercellular bone marrow that cannot be attributed to nutritional deficiency, chronic infection, or
other
chronic systemic illness, and cannot be reversed by the successful treatment of those conditions. Whereas the cases of secondary MDS occur at all ages. Primary MDS is essentially a disease of elderly, though no age group is exempt and predomonant
in
men. The MDS is fundamentally clonal disorders at the level of the haemopoietic totipotent stem cell. Clinically, they are characterized by varing degress of peripheral cytopenia(s) with morphological and functional abnormalities of blood
elements.
We have experienced two unrelated pediatric cases, who showed pancytopenia on admission and the characteristic marow findings of MDS. The first case, a 12 months-old girl, showed increased reticulin with abnormal localization of immature
precursor
cells
(ALIP) in her bone marrow, in addition to dysplastic features such as bi-and multi-nucleation of late normoblasts and internuclear bridging. She also showed 22% of blasts in her bone marrow cells thus classified in the category of RAEB-t after
FAB
classification. Also found were a chromosomal abnormality namely 46 XXt914q21q), which was an unusual one. Her family brought her home and, 4 month later, she deceased. The second case, a 12 year-old boy, showed pancytopenia with
Pseudo-Pelger-Huet
anomaly in neutrophils but no blasts in the peripheral blood. Bone marrow of this case also showed the picture of dyserythropoiesis with binuclearity, internuclear bridging, nuclear fragments, multinuclearity of late normoblasts, in addition to
megaloblastoid change in the normoblasts and increased mitosis in granulocytic series. No chromosomal abnormality was found. Classified as RA after FAB classification of MDS. Showed good response to conservation management for anemia for more
than
one
year.
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