DONOR FOR BMT WITH HEMOGLOBIN-H DISEASE

We report a successful allogeneic BMT for the treatment of juvenile ch ronic myelogenous leukemia (JCML) in a 9-month-old Laotian boy using a n HLA-matched sibling donor with HbH disease (--SEA/alphaalpha(CS)). I n addition, before BMT the recipient had a complex haemoglobinopathy a ssociating heterozygous state AE along with HbH disease (--SEA/-alpha3 ,7) Without haemoglobin Constant Spring (HbCS). Because various haemog lobinopathies are frequently encountered in southeast Asia. when BMT i s performed in Asian families the results may be evaluated by the diff ering haemoglobin characteristics of recipient and donor. However, the re is also a significant risk of transmitting a new haemoglobinopathy to the recipient. Because transplantation from HLA-identical siblings offers the only chance of cure for JCML, the presence of HbH disease w ith mild clinical expression in the donor should not be taken as a con tra-indication to BMT.