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Erythroleukemia of childhood and infancy: a report of two cases
1Department of Pathology, Medical College of Georgia, Augusta 30912, USA.
Insights
This study presents two rare pediatric erythroleukemia (AML-M6) cases in African-American children. Findings correlate bone marrow morphology with immunophenotypes and karyotypes, suggesting distinct pediatric disease presentations.
Area of Science:
- Hematology
- Pediatric Oncology
- Cancer Genetics
Background:
- Erythroleukemia (EL), classified as AML-M6, is a rare subtype of acute myeloid leukemia.
- Pediatric EL cases are exceptionally rare, particularly de novo occurrences.
Observation:
- Two pediatric patients, a 4-month-old and a 4-year-old African-American female, presented with de novo erythroleukemia.
- Clinical presentation included flu-like symptoms and hepatosplenomegaly.
- Peripheral blood revealed anemia and thrombocytopenia.
Findings:
- Bone marrow analysis showed a high percentage of erythroblasts (75-76.8%) with myelodysplastic changes.
- Cytochemical analysis revealed periodic acid-Schiff positivity in erythroblasts.
- Immunophenotyping identified glycophorin-A, CD71, and CD11b positivity.
- Karyotyping demonstrated complex cytogenetic abnormalities in both patients.
Implications:
- The study correlates bone marrow morphology with immunophenotypes and karyotypes in pediatric EL.
- Findings suggest that the initial presentation and disease course in pediatric EL differ from adult forms.
- Laboratory findings in advanced adult EL may resemble those in pediatric EL initial presentation.
Abstract:
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.