Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 from 1989 to 1995. The clinical, morphologic, immunophenotypic and cytogenetic features of both patients are reviewed. The purpose of this study is to correlate the bone marrow morphology with the immunophenotypes and the karyotypes of the neoplastic cells. The patients were both female, presented with flu-like symptoms, and were noted to have hepatosplenomegaly on physical examination. The peripheral blood examination was significant for anemia (Hb 54 (A), 84(B)g/L), and thrombocytopenia (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 glycophorin-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 different in adults and children. However, once the adult form reaches the acute leukemia stage, the laboratory findings are similar to those at initial presentation in pediatric EL.
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PMID:Erythroleukemia of childhood and infancy: a report of two cases. 909 14