Journal of the Korean Pediatric Society 1999;42(7):1003-1007.
Published online July 15, 1999.
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
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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