Table of Contents

HK J Paediatr (New Series)
Vol 19. No. 1, 2014

HK J Paediatr (New Series) 2014;19:32-36

Case Report

Unrelated Cord Blood Transplantation in a Pair of Siblings with X-linked Severe Combined Immunodeficiency: The Case for Early Diagnosis and Transplant

PYT Chan, MHK Ho, DKL Cheuk, TL Lee, SY Ha, YL Lau


Haematopoietic stem cell transplantation is a curative therapy for severe combined immunodeficiency (SCID) after the first success forty years ago. We report a pair of Chinese siblings with X-linked SCID who had very different transplant outcomes. The elder brother was diagnosed late and had multiple infections before transplant. He developed severe graft-versus- host- disease and life-threatening haemolytic anaemia. He died from overwhelming septicaemia. The younger brother, who was diagnosed at birth and infection-free before transplant, underwent an uneventful transplant. Early transplant within the first 3 months of life results in over 90% disease-free survival. Newborn screening is now being pursued to achieve this goal.

Keyword : Haematopoietic stem cell transplantation; Haemolytic anaemia; Pneumatosis intestinalis; Severe combined immunodeficiency; Transplant outcome

Abstract in Chinese


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