DIAMOND BLACKFAN ANEMIA: A TERTIARY CARE CENTRE EXPERIENCE

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avinash kumar singh
tulika seth *
nita radhakrishnan
pravas mishra
manoranjan mahapatra
haraprasad pati
(*) Corresponding Author:
tulika seth | tuliseth@yahoo.com

Abstract

Introduction: Diamond Blackfan anemia (DBA) is a rare congenital hypoplastic anemia that usually presents early in infancy. Methods: All consecutive children of DBA attending the Department of Hematology OPD, AIIMS were included in the study. This is a retrospective study, with few cases enrolled prospectively, over the last 3 years. Aim: To study the clinical profile & treatment response of Diamond Blackfan anemia patients at our center. Results: 10 patients were included in the study; male: female ratio was 9:1. Median age was 2.5 yrs, ranging from 4 months to 8 yrs. Anemia requiring frequent blood transfusions was the predominant complaint since infancy. Two children were siblings.Abnormal phenotypic features were observed in 60%. The craniofacial abnormalities seen included triangular facies with DBA phenotype 50 % (5), microcephaly 10 %( 1), low set ears 10 %( 1), low hairline 10 %( 1), wide spaced teeth 10%( 1), malar prominence 10 %( 1), wide spaced toes 10 %( 1), growth failure 30%( 3) and wide spaced nipple10 % (1). Out of 10 patients, 5(50%) are transfusion independent on steroids, 4 patients had partial response with steroids and 1 had partial response on cyclosporine with occasional transfusions and the other 3 are transfusion dependent and on iron chelation. Conclusion: DBA is a rare congenital hypoplastic anemia. About 50% of patients have a good response to steroids.


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Author Biographies

avinash kumar singh, ALL INDIA INSTITUTE OF MEDICAL SCIENCE

Department of HematologySeniour Resident

tulika seth, Department of Hematology,AIIMS,Delhi,India

M.D.

nita radhakrishnan, Department of Hematology,AIIMS,Delhi,India

M.D.

pravas mishra, Department of Hematology,AIIMS,Delhi,India

M.D.,D.M.

manoranjan mahapatra, Department of Hematology,AIIMS,Delhi,India

M.D.

haraprasad pati, Department of Hematology,AIIMS,Delhi,India

M.D.

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