All issues > Volume 40(3); 1997

Case Report

J Korean Pediatr Soc. 1997;40(3):417-422. Published online March 15, 1997.

A Case of Granular Acute Lymphoblastic Leukemia with t(5p;5p) Arising in Down Syndrome Infant

Soon Ja SJ Lee¹, Young Ho YH An¹, Hai Lee HL Chung¹

¹Department of Pediatrics School of Medicine, Catholic University of Taegu Hyosung, Taegu, Korea

Abstract

Granular lymphoblast which is characterized by the presence of clearly defined azurophilic cytoplasmic granules are a relatively uncommon finding and indicate a negative impact on prognosis of childhood ALL. Granular ALL is more common in FAB L2 cases but there is no significant difference by immunophenotype and no specific cytogenetic abnormality correlated with clinical significance of granular ALL has been reported. We present a case of granular acute lymphoblastic leukemia arising in a 18 month old infant with Down syndrome. More than 60% of marrow lymphoblasts contain large azurophilic granules in cytoplasm, which were stained negative for myeloperoxidase, SBB, NSE, and positive for PAS and acid phosphatase. Our case was identified as T-cell leukemia by immunophenotyping. The result of chromosome study on marrow blasts at diagnosis was 47, XY, +21, t(5p;5p) and showed chromosomalrearrangement during the course of disease.

Keywords :granular acute lymphoblastic leukemia cytogenic abnormality