Korean J Hematol 2000; 35(1):

Published online March 31, 2000

© The Korean Society of Hematology

소아 골수이형성 증후군의 혈액학적 소견

최수진, 박찬정, 서을주, 지현숙, 서종진, 김태형, 문형남

울산대학교 의과대학 서울중앙병원 임상병리과,
울산대학교 의과대학 서울중앙병원 소아과

Hematologic Findings of Childhood Myelodysplastic Syndrome

Soo Jin Choi, Chan Jeoung Park, Eul Zu Seo, Hyun Sook Chi, Jong Jin Seo, Tad Ghim, Hyung Nam Moon

Department of Clinical Pathology, Pediatrics, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea

Abstract

BACKGROUND: The myelodysplastic syndrome (MDS) in childhood is considered to be very rare and the nature of this disease in childhood is very different in from that in adults. We analyzed the hematologic findings of childhood myelodysplastic syndrome.
METHODS: The retrospective study was performed with the confirmed patients who were admitted to the Department of Pediatrics, Asan Medical Center (AMC) from June 1989 till May 1999, to analyzed the hematologic findings. Sixteen children with a primary myelodysplastic syndrome (MDS) were presented to AMC during a 10 year period.
RESULTS: Morphological assessment of the peripheral blood and the bone marrow showed nine patients (56%) had refractory anemia (RA), two patients (13%) had RA with excess blasts (RAEB), five patients (31%) had RAEB in transformation (RAEB-t). Five children with juvenile chronic myelogenous leukemia were diagnosed over the same period. Age distribution showed the predilection between 6∼10 tears and male : female ratio was 1:1. In peripheralbood, pancytopenia was found in seven cases (44%) and leukocytosis in four cases (25%). The bone marrow findings showed hypercellularity in 54%, hypocellularity in 8% and variable cellularity in 15% and myelofibrosis was observed in 23%. Various dyspoietic changes of erythrocytes, leukocytes and platelets
in peripheral blood and three cell lines in bone marrow were observed and trilineage dysplasia was observed in 62% of MDS, and 56% of RA. The four cases (25%) of RAEB and RAEB-t have transformed to acute myelogenous leukemia.
CONCLUSION: In our study, the MDS in childhood seemed to be characterized by higher incidence of RA. Compared with the adult MDS, trilineage dysplasia in RA was frequently seen, but, no significant differences of dyspoietic features between adults and childhood were observed.

Keywords Myelodysplastic syndrome; Childhood; Hematologic findings;

Article

Korean J Hematol 2000; 35(1): 21-26

Published online March 31, 2000

Copyright © The Korean Society of Hematology.

소아 골수이형성 증후군의 혈액학적 소견

최수진, 박찬정, 서을주, 지현숙, 서종진, 김태형, 문형남

울산대학교 의과대학 서울중앙병원 임상병리과,
울산대학교 의과대학 서울중앙병원 소아과

Hematologic Findings of Childhood Myelodysplastic Syndrome

Soo Jin Choi, Chan Jeoung Park, Eul Zu Seo, Hyun Sook Chi, Jong Jin Seo, Tad Ghim, Hyung Nam Moon

Department of Clinical Pathology, Pediatrics, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea

Abstract

BACKGROUND: The myelodysplastic syndrome (MDS) in childhood is considered to be very rare and the nature of this disease in childhood is very different in from that in adults. We analyzed the hematologic findings of childhood myelodysplastic syndrome.
METHODS: The retrospective study was performed with the confirmed patients who were admitted to the Department of Pediatrics, Asan Medical Center (AMC) from June 1989 till May 1999, to analyzed the hematologic findings. Sixteen children with a primary myelodysplastic syndrome (MDS) were presented to AMC during a 10 year period.
RESULTS: Morphological assessment of the peripheral blood and the bone marrow showed nine patients (56%) had refractory anemia (RA), two patients (13%) had RA with excess blasts (RAEB), five patients (31%) had RAEB in transformation (RAEB-t). Five children with juvenile chronic myelogenous leukemia were diagnosed over the same period. Age distribution showed the predilection between 6∼10 tears and male : female ratio was 1:1. In peripheralbood, pancytopenia was found in seven cases (44%) and leukocytosis in four cases (25%). The bone marrow findings showed hypercellularity in 54%, hypocellularity in 8% and variable cellularity in 15% and myelofibrosis was observed in 23%. Various dyspoietic changes of erythrocytes, leukocytes and platelets
in peripheral blood and three cell lines in bone marrow were observed and trilineage dysplasia was observed in 62% of MDS, and 56% of RA. The four cases (25%) of RAEB and RAEB-t have transformed to acute myelogenous leukemia.
CONCLUSION: In our study, the MDS in childhood seemed to be characterized by higher incidence of RA. Compared with the adult MDS, trilineage dysplasia in RA was frequently seen, but, no significant differences of dyspoietic features between adults and childhood were observed.

Keywords: Myelodysplastic syndrome, Childhood, Hematologic findings,

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