Br Hawkins

Successful mismatched sibling cord blood transplant in Hb Bart's disease

A 20-month-old girl with Hb Barts disease, who had survived neonatal complications, underwent HLA-DR antigen mismatched sibling cord blood transplantation successfully. Immune thrombocytopenia, which occurred around 2.5 months after transplant, responded to intravenous γ-globulin. The fetal hemoglobin level rose to a peak of 52.3% on day +69 post transplant and declined gradually during the following year. Ten percent of hemoglobin Barts was detected 2 months after transplant and this reflects the α-thalassemia trait of the donor. Bone Marrow Transplantation (2001) 28, 105–107.

Association of the HLA A2-B46-DR9 haplotype with autoimmune thyroid dysfunction after bone marrow transplantation in Chinese patients.

Autoimmune thyroid disease (AITD) may occur in patients after bone marrow transplantation (BMT). At a median follow‐up of 4 years, among 194 allografts and 28 autografts, four patients (three allografts, one autograft) developed AITD. All carried the human leucocyte antigen (HLA) A2‐B46‐DR9 haplotype, strongly associated with AITD in the Chinese population. No significant thyroid disorder was detected in 190 patients without this haplotype. The frequency of AITD in BMT patients with the HLA A2‐B46‐DR9 haplotype was 12·5%, with a relative risk of 7·8 times that of non‐carriers (P < 0·001). The risk of AITD should be recognized in recipients with high‐risk HLA haplotypes, and regular screening might be warranted.

Human leucocyte antigens in Southern Chinese with Kawasaki disease

Sir: Several invest igators have previously shown an associat ion of cer ta in h u m a n leucocyte ant igens ( H L A ) with Kawasaki disease including H L A B w 2 2 in Japanese [1] and H L A B 5 1 in Caucasians [3]. To date the re have b e e n no repor ted investigat ions of the possible associat ion of H L A ant igens with Kawasaki disease in Chinese chi ldren. We the re fo re looked for an H L A associat ion in 62 pa t ien ts of Sou the rn Chinese origin living in H o n g Kong. A tota l of 62 pa t ien ts (38 males, 24 females) were diagnosed as having Kawasaki disease according to the diagnost ic cri teria of the J apan Kawasaki Disease Resea rch Commi t t ee [6]. Cont ro ls consisted of 100 normal adults selected on the basis of thei r origin in Sou the rn China or H o n g Kong. H L A typing for H L A A , -B and D R ant igens was pe r fo rmed using a s tandard lymphocyte microcytotoxici ty technique . Statistical analysis was by chi-square test. The results are shown in Table 1. None of the H L A A , -B or D R ant igens in our pa t ien ts showed significant differences in p reva lence compared with normal controls . This is in contras t to previous findings in o the r e thnic groups. Kato et al. [1] found a significant increase in the f requency of H L A B w 2 2 in a group of 205 Japanese pat ients compared with no rma l controls (25.4% versus 11.8%) a l though Ma t suda et al. [5] had found no significant difference f rom controls in 32 Japanese pat ients . In our s tudy of 62 pat ients, the f requency of H L A B w 2 2 was similar in pa t ien ts and controls , even w h e n the ant igen was split in to its subtypes of HLA-Bw54, -Bw55, and -Bw56 (data not shown). In Caucasians, a study of 23 pat ients in Boston, Un i t ed States of Amer ica [3] showed a significant associat ion of H L A B 51 with Kawasaki disease wi th a relat ive risk of 14.8. H L A B 51 has also b e e n shown to be associated with the disease in 12 Jewish Israeli chi ldren [2]. In our s tudy the re was no significant difference be tween pa t ien ts and controls in the f requency of H L A B51 or of any o the r H L A B 5 sub-group (data no t shown). Krensky et al. [4] in a study of 51 Caucas ian pat ients with Kawasaki disease showed that 80% of patients presenting during an endemic per iod were H L A B 5 (70% HLA-Bw51) whereas there were no H L A B 5 individuals dur ing an epidemic period. The re was, however , an increased preva lence of H L A B w 4 4 dur ing the epidemic [4]. All of our cases were endemic and no s t a t emen t could be drawn on the dis t r ibut ion of H L A B w 4 4 in our study. In conclusion we did not find any associat ion of H L A antigen with Kawasaki disease in Sou the rn Chinese .