RESUMO
Erythroleukemia is an uncommon disorder in children. Four cases of pediatric erythroleukemia, diagnosed over a period of nine years are presented. The patients presented with pallor, fever and hepatosplenomegaly of recent onset. Peripheral smear examination showed anemia, thrombocytopenia and circulating blasts. The bone marrow displayed erythroid hyperplasia with dysplasia and PAS positive erythroblasts. Myeloid blasts were myeloperoxidase positive and one case showed positivity for non specific esterase, indicating monocytoid differentiation, a poor prognostic feature. Prognosis was poor and follow up period was short.
Assuntos
Criança , Feminino , Testes Hematológicos , Humanos , Leucemia Eritroblástica Aguda/complicações , MasculinoRESUMO
We report the case of a three year old female child with combined occurrence of von Willebrand's disease and factor XIII deficiency, an extremely rare combination. The patient presented with prolonged bleeding following cuts and wounds. Clot solubility test using 5M urea was positive. Platelet aggregation using ristocetin was reduced, which corrected on adding normal plasma. Aggregation with other agonists was normal. We discuss the clinico- hematological profile of the case. Only one such case has been reported in literature in the past to the best of our knowledge.