RESUMO

A case of dyserythropoietic congenital anemia type II (HEMPAS) in an infant 35 days of age is reported. Diagnosis was based upon morphological features of the erythroblasts, 25% of them were bi or multinucleated and a positive acidified serum test (pseudo HAM). The early and severe anemia which was present from the first days of life is emphasized. An additional and significant hemolytic factor with a relative tocopherol deficiency is suggested. A very important degree of hepatic hemosiderosis was found at necropsy. This finding reveals the very active dyserytropietic disorder in this case from the beginning of intrauterine file.

Assuntos

Anemia Hemolítica Congênita/patologia , Hemossiderose/patologia , Doenças do Recém-Nascido/patologia , Talassemia/patologia , Diagnóstico Diferencial , Eritroblastos/patologia , Humanos , Lactente , Recém-Nascido , Fígado/patologia