All issues > Volume 42(7); 1999

Case Report

J Korean Pediatr Soc. 1999;42(7):1003-1007. Published online July 15, 1999.

A Case of Ectopic CD7(+) Congenital Monocytic Leukemia

Na Yeon NY Kim¹, Dong Seok DS Lee¹, Jin Hwa JH Jeong¹, Sung Min SM Cho¹, Doo Kwun DK Kim¹, Sung Min SM Choi¹, Woo Taek WT Kim¹, Kyung Im KI Ha²

¹Department of Pediatrics, School of Medicine, Dongguk University, Kyongju, Korea
²Department of Clinical Pathology, School of Medicine, Dongguk University, Kyongju, Korea

Correspondence Na Yeon NY 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.

Keywords :CD7(＋) Congenital monocytic leukemia, CNS involvement, Chromosomal anomaly