A Case of Ectopic CD7(+) Congenital Monocytic Leukemia |
Na Yeon Kim1, Dong Seok Lee1, Jin Hwa Jeong1, Sung Min Cho1, Doo Kwun Kim1, Sung Min Choi1, Woo Taek Kim1, Kyung Im Ha2 |
1Department of Pediatrics, School of Medicine, Dongguk University, Kyongju, Korea 2Department of Clinical Pathology, School of Medicine, Dongguk University, Kyongju, Korea |
이소성 림프구 표지자 CD7을 발현한 선천성 급성 단구성 백혈병 1례 |
김나연1, 이동석1, 정진화1, 조성민1, 김두권1, 최성민1, 김우택1, 하경임2 |
1동국대학교 의과대학 소아과학교실 2동국대학교 의과대학 임상병리학교실 |
Correspondence:
Na Yeon Kim, Email: 1 |
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Abstract |
Congenital acute leukemia is a rare disorder with approximately 200 cases reported. It is defined as a childhood leukemia occurring at birth or before 1 month of age at a rate of 1%. Acute leukemias are generally classified according to morphology, cytochemistry and cell surface marker expression. Most leukemias conform to an ordered lineage-specific pattern of gene expression, but a small subset of leukemias appears not to follow lineage restriction. Several reports revealed a subgroup of acute myelogenous leukemia(AML) that expresses CD7, a cell surface marker expressed early during T lineage differentiation, especially in less differentiated AML subtypes. We report a rare case of CD7(+) congenital monocytic leukemia(M5a) with detailed immunophenotypic and cytochemical characterization in an 8 week-old female. She had central nervous system (CNS) involvement at diagnosis. Chromosomal analysis revealed a mosaicism with 46,XX,-6,de1(7) (q21),t(19;21)(q13.3;q22)/46,XX, that has not been reported. |
Key Words:
CD7(+) Congenital monocytic leukemia, CNS involvement, Chromosomal anomaly |
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