ERYTHROLEUKEMIA OF CHILDHOOD AND INFANCY - A REPORT OF 2 CASES

Two rare de novo cases are presented of pediatric erythroleukemia (EL) , AML-M6, in a four-month-old (patient A) and four-year-old (patient B ) African-Americans who presented to the Medical College of Georgia fr om 1989 to 1995. The clinical, morphologic, immunophenotypic, and cyto genetic features of both patients are reviewed. The purpose of this st udy is to correlate the bone marrow morphology with the immunophenotyp es and the karyotypes of the neoplastic cells. The patients were both female, presented with flu-like symptoms, and were noted to have hepat osplenomegaly on physical examination. The peripheral blood examinatio n was significant for anemia (Hb 54 (A), 84(B)g/L), and thrombocytopen ia (86 (A), 70(B) X 10(9)/L). The bone marrow contained 75 percent (A) and 76.8 percent (B) erythroblasts and showed myelodysplastic changes in the erythroid cell line. Cytochemical analysis was performed, and greater than 10 erythroblasts per 100 cells were periodic acid-Schiff positive. Immunophenotypes of the pretreatment bone marrow showed glyc ophorin-A, CD71, and CD11b positivity. The karyotypes of both patients contained complex (>3 per clone) cytogenetic abnormalities. Our data suggest that the initial presentation and course of disease are differ ent in adults and children. However, once the adult form reaches the a cute leukemia stage, the laboratory findings are similar to those at i nitial presentation in pediatric EL.