Korean J Hematol 2000; 35(2):

Published online June 30, 2000

© The Korean Society of Hematology

6개월 영아에서 발병한 연소형 골수단구성백혈병 1예

장은아, 김문규, 임영애, 현봉학, 김신원, 박광화, 홍정

아주대학교 의과대학 임상병리학교실,
아주대학교 의과대학 소아과학교실,
아주대학교 의과대학 해부병리학교실,
아주대학교 의과대학 일반외과학교실

A Case of Juvenile Myelomonocytic Leukemia in a Six Months Old Infant

Eun Ah Chang, Moon Kyu Kim, Young Ae Lim, Bong Hak Hyun, Shin Won Kim, Kwang Hwa Park, Jeong Hong

Department of Clinical Pathology, Pediatrics, Anatomical Pathology, General Surgery, College of Medicine, Ajou University, Suwon, Korea

Abstract

Myelodysplastic syndrome (MDS) in childhood is a rare hematologic malignancy and its classification has been the subject of some controversy. Cases of pediatric MDS are subdivided into those with features of adult-type MDS and those with myeloproliferative features occasionally observed in infancy and early childhood. There appears to be an international consensus to rename the disease juvenile myelomonocytic leukemia (JMML), which includes all leukemias of childhood previously classed as chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia(JCML), and infantile monosomy 7 syndrome. We experienced a 6-month-old female infant with JMML who developed extensive extramedullary hematopoiesis. The patient developed abdominal distention, hepatosplenomegaly, anemia, thrombocytopenia, and leukocytosis with significant monocytosis and was found to have a high hemoglobin F level of 30%. Her bone marrow biopsy section and aspirate smears revealed normocellularity with no increment of blast cells and no dysplastic changes. Cytogenetic analysis revealed a normal 46, XX karyotype. Her liver, spleen, lymphnodes, and appendix were found to be heavily infiltrated by partially differentiated myelomonocytic cells. These findings supported the diagnosis of JMML with extensive extramedullary hematopoiesis.

Keywords Juvenile myelomonocytic leukemia; Childhood myelodysplastic syndrome; Fetal hemoglobin; Infant; Extramedullary hematopoiesis;

Article

Korean J Hematol 2000; 35(2): 189-194

Published online June 30, 2000

Copyright © The Korean Society of Hematology.

6개월 영아에서 발병한 연소형 골수단구성백혈병 1예

장은아, 김문규, 임영애, 현봉학, 김신원, 박광화, 홍정

아주대학교 의과대학 임상병리학교실,
아주대학교 의과대학 소아과학교실,
아주대학교 의과대학 해부병리학교실,
아주대학교 의과대학 일반외과학교실

A Case of Juvenile Myelomonocytic Leukemia in a Six Months Old Infant

Eun Ah Chang, Moon Kyu Kim, Young Ae Lim, Bong Hak Hyun, Shin Won Kim, Kwang Hwa Park, Jeong Hong

Department of Clinical Pathology, Pediatrics, Anatomical Pathology, General Surgery, College of Medicine, Ajou University, Suwon, Korea

Abstract

Myelodysplastic syndrome (MDS) in childhood is a rare hematologic malignancy and its classification has been the subject of some controversy. Cases of pediatric MDS are subdivided into those with features of adult-type MDS and those with myeloproliferative features occasionally observed in infancy and early childhood. There appears to be an international consensus to rename the disease juvenile myelomonocytic leukemia (JMML), which includes all leukemias of childhood previously classed as chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia(JCML), and infantile monosomy 7 syndrome. We experienced a 6-month-old female infant with JMML who developed extensive extramedullary hematopoiesis. The patient developed abdominal distention, hepatosplenomegaly, anemia, thrombocytopenia, and leukocytosis with significant monocytosis and was found to have a high hemoglobin F level of 30%. Her bone marrow biopsy section and aspirate smears revealed normocellularity with no increment of blast cells and no dysplastic changes. Cytogenetic analysis revealed a normal 46, XX karyotype. Her liver, spleen, lymphnodes, and appendix were found to be heavily infiltrated by partially differentiated myelomonocytic cells. These findings supported the diagnosis of JMML with extensive extramedullary hematopoiesis.

Keywords: Juvenile myelomonocytic leukemia, Childhood myelodysplastic syndrome, Fetal hemoglobin, Infant, Extramedullary hematopoiesis,

Blood Res
Volume 59 2024

Stats or Metrics

Share this article on

  • line

Blood Research

pISSN 2287-979X
eISSN 2288-0011
qr-code Download