Bone marrow transplant cure for beta-thalassaemia major: initial experience from a developing country.

Ann Hematol 2008 Aug 6;87(8):655-61. Epub 2008 May 6.

Department of Haematology, Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Punjab, Pakistan.

Between July 2001 and June 2007, 48 consecutive patients with beta-thalassaemia major received allogeneic haematopoietic stem cell transplants (allo HSCT) from human-leukocyte-antigen-matched siblings at the Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan, using standard conditioning regimens. The median age of the patient cohort was 4 years (range, 1-14 years). Thirty-one patients were in risk class I, 11 in class II and six patients were in class III. Engraftment was achieved in all patients. Survival was calculated from the date of transplant to death or last follow-up. Major post-transplant complications encountered were acute graft versus host disease (Ac GvHD) (grades II-IV), 35.4%; chronic GvHD, 8.3%; haemorrhagic cystitis, 12.5%; veno-occlusive disease (VOD) of the liver, 6.2%; bacterial infections, 37.5%; fungal infections, 19%; cytomegalovirus (CMV) infection, 6.2%; herpes infection, 6.2%; and tuberculosis in 2% of patients. Graft rejection was observed in five patients. Three patients received second transplants. Mortality was observed in 20.8% of patients. Major fatal complications included GvHD, VOD, intracranial haemorrhage, septicaema, CMV disease and disseminated tuberculosis. Overall survival and disease-free survival were 79% and 75%, respectively, at 6 years post-HSCT.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00277-008-0478-8DOI Listing
August 2008

Publication Analysis

Top Keywords

patients
8
infection 62%
8
marrow transplant
8
beta-thalassaemia major
8
bone marrow
8
class iii
4
62% herpes
4
patients class
4
class class
4
class patients
4
iii engraftment
4
cytomegalovirus cmv
4
patients survival
4
survival calculated
4
achieved patients
4
engraftment achieved
4
cmv infection
4
risk class
4
calculated transplant
4
thirty-one patients
4

Similar Publications