[Myelodysplasia with monosomia 7 in child. study about 2 observations treated by backbone transplant]

ABSTRACT

The myelodysplasia with monosomia 7 is a rare clonal haemopathy in child. Its characterized by defect of haematopofetic souche cell maturation explaining the board of the rich backbone cytopenia. The severe prognosis of this affectiOn [death linked to the medullar deficiency or to the refractory myeloblastic acute leukaemia] justify the backbone transplant if there is an intra-familial blood donor. The other therapeutical approaches remain experimental. We report two observations of myelodysplasia with monosomia 7 in two girls aged of 8 years old and a half and 10 years revealed by cutaneous palor without tumoral syndrome. The biological balance revealed a pancytopenia in one case and an isolated macrocytary anaemia in the other case. The myelogram confirmed the myelodysplasia diagnosis providing a riche marrow with presence of dyserythropofesis signs. The medular caryotype confirmed the monosomia 7 by revealing a chromosomic cellular clone 45 XX-7. The backbone transplant practized in these two girls permetted to normalize the haemogram and to disappear the chromosomic anomalies in the one from them. The second is dead in the 73rd day following the graft reaction against the host and a cytomegalovirus digestive infection