| A Case of Granular Acute Lymphoblastic Leukemia with t(5p;5p) Arising in Down Syndrome Infant |
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Soon Ja Lee, Young Ho An, Hai Lee Chung |
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Department of Pediatrics School of Medicine, Catholic University of Taegu Hyosung, Taegu, Korea |
| 다운 증후군 환아에서 t(5p;5p)를 보인 과립성 급성 림프구성 백혈병 1례 |
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이순자, 안영호, 정혜리 |
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대구효성가톨릭대학교 의과대학 소아과학교실 |
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| Abstract |
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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. |
| Key Words:
granular acute lymphoblastic leukemia cytogenic abnormality |
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