J Korean Pediatr Soc.  1997 Mar;40(3):417-422.

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

• Lee SJ
• An YH
• Chung HL

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 chromosomal rearrangement during the course of disease.

Keyword

granular acute lymphoblastic leukemia cytogenic abnormality

MeSH Terms

Acid Phosphatase
Bone Marrow
Chromosome Aberrations
Cytoplasm
Cytoplasmic Granules
Diagnosis
Down Syndrome*
Humans
Immunophenotyping
Infant*
Leukemia, T-Cell
Peroxidase
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
Prognosis
Acid Phosphatase
Peroxidase