Razi Journal of Medical Sciences

    Although leukemia is the most common malignancy in childhood, congenital leukemia which manifests itself within the first 4 weeks of life is rare and accounts for less than 1% of all leukemias in childhood. Congenital leukemia should be differentiated from transient myeloproliferative disorder(TMD) which is noted in Down Syndrome. Among the reported patients, acute myeloid leukemia(AML) was more frequent(64%) than acute lymphoblastic leukemia(ALL 21%). Cytogenetic abnormality was found in 72%, 42% of which was 11q23 abnormality. The survival of AML patients was better than ALL. Until now, 18 cases of congenital leukemia showing spontaneous remission have been described and all of them were AML(FAB M5, M4). Clinical characteristics of congenital leukemia differ from those of leukemia in older children, the outcome is poor, and its chemotherapy needs improvement to increase the remission rate. In the present article, we report an 18-day-old female with congenital leukemia who had blueish infiltrative lesion at different parts of skin, jaundice, hepatosplenomegaly, anemia, and thrombocytopenia hyperleukocytosis from the birth. Peripheral blood smear and bone marrow aspiration showed ALL L1. Immunophenotype evaluation showed undifferentiated leukemia, and skin biopsy revealed infiltration of leukemia(leukemia cutis).