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Clinical and hematologic manifestations in patients with diamond blackfan anemia in korea

Title
Clinical and hematologic manifestations in patients with diamond blackfan anemia in korea
Authors
Kim S.-K.Ahn H.-S.Kang H.-J.Kim H.Park K.-D.Shin H.-Y.Back H.-J.Kook H.Cho B.Chung N.-G.Jeoung D.-C.Choi E.-J.Hwang P.-H.Ko K.-N.Lim H.-J.Park S.-K.Seo J.-J.Koo H.-H.Sung K.-W.Lee K.-C.Lim Y.-T.Lyu C.-J.Park J.-E.Ryu K.-H.Yoo E.S..
Ewha Authors
유경하유은선
SCOPUS Author ID
유경하scopus; 유은선scopus
Issue Date
2012
Journal Title
Korean Journal of Hematology
ISSN
1738-7949JCR Link
Citation
vol. 47, no. 2, pp. 131 - 135
Indexed
SCOPUS scopus
Abstract
Background Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rarecondition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. Methodsm The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. Results The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1±1.9 g/dL, mean corpuscular volume was 93.4±11.6 fL, and mean number of reticulocytes was 19,700/mm 3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). Conclusion The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses. © 2012 Korean Society of Hematology.
DOI
10.5045/kjh.2012.47.2.131
Appears in Collections:
의학전문대학원 > 의학과 > Journal papers
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