PMID- 19494396
OWN - NLM
STAT- MEDLINE
DCOM- 20090814
LR  - 20161020
IS  - 1024-2708 (Print)
IS  - 1024-2708 (Linking)
VI  - 15
IP  - 3 Suppl 3
DP  - 2009 Jun
TI  - Haematopoietic stem cell transplantation for thalassaemia in Chinese patients.
PG  - 39-41
AB  - Beta thalassaemia major is a common hereditary haematological disease in southern 
      Chinese. Advances in transfusion and iron chelation improve survival but 
      haematopoietic stem cell transplantation (HSCT) is still the only curative 
      treatment. Due to repeated blood transfusion and iron overload, thalassaemia 
      patients undergoing HSCT are at a higher risk of graft rejection and 
      transplant-related mortality. The prognostic factors identified to be affecting 
      transplant outcome include hepatomegaly, hepatic fibrosis, and compliance to 
      chelation therapy. Patients can be classified into three classes and conditioning 
      regimens are modified according to the risk. Early stage patients have 85 to 90% 
      chance of disease-free survival, whereas advance stage only has 60% disease-free 
      survival. Mixed chimerism is common after HSCT but majority have satisfactory 
      erythropoiesis without need for further transfusion. Sibling cord blood and bone 
      marrow transplantation has similar outcome. Recently alternative donor transplant 
      has been performed in patients without human leukocyte antigen (HLA)-identical 
      siblings. The result of unrelated-donor bone marrow transplantation is in general 
      inferior but extended HLA matching may improve outcome. The use of unrelated cord 
      blood transplant from a single-centre study showed promising result. The 
      survivors require iron depletion to remove excessive iron store and some may 
      require hormonal replacement therapy. Most of the patients have good quality of 
      life after successful HSCT.
FAU - Li, C K
AU  - Li CK
AD  - Department of Paediatrics, Prince of Wales Hospital, The Chinese University of 
      Hong Kong, Shatin, Hong Kong. ckli@cuhk.edu.hk
FAU - Lee, Vincent
AU  - Lee V
FAU - Shing, Matthew M K
AU  - Shing MM
FAU - Leung, T F
AU  - Leung TF
LA  - eng
PT  - Journal Article
PL  - China
TA  - Hong Kong Med J
JT  - Hong Kong medical journal = Xianggang yi xue za zhi
JID - 9512509
SB  - IM
MH  - China
MH  - Disease-Free Survival
MH  - Hematopoietic Stem Cell Transplantation/*ethnology
MH  - Humans
MH  - Thalassemia/*ethnology/*therapy
MH  - Transplantation Conditioning/*methods
MH  - Transplantation, Homologous
EDAT- 2009/06/25 09:00
MHDA- 2009/08/15 09:00
CRDT- 2009/06/05 09:00
PHST- 2009/06/05 09:00 [entrez]
PHST- 2009/06/25 09:00 [pubmed]
PHST- 2009/08/15 09:00 [medline]
PST - ppublish
SO  - Hong Kong Med J. 2009 Jun;15(3 Suppl 3):39-41.