exudates and Graft-vs-Host-Disease

Inborn errors of immunity (IEI) comprise a heterogeneous group of disorders of the immune system, most of which are curable by haematopoietic stem cell transplantation (HSCT). We present a 25-year audit of HSCT for IEI at a tertiary-level academic hospital in Malaysia.. Review of medical records of all cases of IEI who underwent HSCT between January 1993 and December 2018 at our centre. Diagnoses, complications, HSCT protocols and outcome data were studied.. There were 20 patients (19 boys) with a median age at diagnosis of 11 months (range: 2 months to 12 years). Eleven of 19 (58%) had malnutrition at presentation. Donor sources were variable: 13 (65%) matched sibling donor (MSD), 4 (20%) human leukocyte antigen-haploidentical donor (HD) and 3 (15%) matched unrelated donor (MUD). Conditioning regimens were physician-dependent and adapted to each patient's clinical status. Grades III-IV acute graft-versus-host disease occurred in two of three cases who received MUD grafts, 50% in those who received HD, and 8% in the MSD group. Transplant-related mortality at day +100 was 5%. With a median follow-up of 7.5 years, 18 (90%) patients are alive and free of infections.. Outcome of HSCT for IEI in our centre is comparable with international reports. HSCT results using HD and MUD grafts are also good despite challenges from acute graft-versus-host disease, providing a feasible alternative for patients without matched donors.

Topics: Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Hospitals; Humans; Malaysia; Male; Siblings; Transplantation Conditioning

Gamma-irradiation of blood components is regarded a safe procedure used for prevention of transfusion associated graft-versus-host disease. However, reports showed that irradiation can cause erythrocyte haemolysis and damage to the RBC membrane. In University Kebangsaan Malaysia Medical Centre (UKMMC), a number of suspected transfusion reactions (TR) featured unusual isolated episodes of red-coloured-urine or haemoglobinuria among paediatric patients without clinical features of acute haemolytic TR. Haemolysis of irradiated red cells was suspected as a cause. This study was conducted to evaluate haemolytic changes of RBC components following irradiation. A prospective, pre- and post- irradiation comparative study was conducted on 36 paired RBC-components in the blood-bank, UKMMC in the year 2013. Samples were tested for plasma-Hb, percent-haemolysis, plasma-potassium (K⁺) and lactate dehydrogenase (LDH) level. Post-irradiation mean plasma-Hb and percent-haemolysis were significantly higher than pre-irradiation values at 0.09 ±0.06g/dl VS 0.10 ± 0.06g/dl and 0.19 ± 0.13% VS 0.22 ± .13% respectively, while plasma-K⁺ and LDH values did not show significant difference. However, the mean percent-haemolysis level was still within recommended acceptable levels for clinical use, supporting that irradiated RBC units were safe and of acceptable quality for transfusion. There was no conclusive reason for isolated haemoglobinuria following transfusion of irradiated red-cell products. Further research is suggested to investigate the other possible causes.

Topics: Blood Component Transfusion; Cross-Sectional Studies; Erythrocytes; Graft vs Host Disease; Hemolysis; Humans; Malaysia; Prospective Studies

Malaysia conducted the first BMT in the country in 1987. Since then, there have been 1155 transplantations performed in a total of eight transplant centres. A majority of the transplantations were allogeneic, including myeloablative and nonmyeloablative. A vast majority of donors are HLA identical siblings. The mean age of transplanted patients was 26 years. The major reason for transplantation was hematological malignancies. The overall survival was 60% for allogeneic transplantation and 52% for autologous transplantation. The most common cause of death in transplanted patients was the underlying disease followed by infection-related complications. Currently, the government is expanding the existing public cord blood bank as well as the local donor registry.

Topics: Adult; Bone Marrow Transplantation; Female; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Malaysia; Male

We analysed the outcome of 104 patients from a single institution who underwent allogeneic haematopoietic stem cell transplantation (AHSCT) from their HLA-identical siblings between 1993 and 2006. Sixty-nine percent of patients had peripheral blood stem cell (PBSC) as the stem cell source and the remaining had bone marrow (BM). The majority of patients are Chinese (60%) followed by Malays (24%) and Indians (14%). The median time to reach white cell counts of >1 x 10(9)/L and platelet counts of >30 x 10(9)/L was 13 and 15 days, respectively in patients who had PBSC transplantation compared with 16 and 25 days in patients who had BM transplantation, (p < 0.0001 and p < 0.001). Acute graft-versus-host disease (aGVHD) of grade II to IV was observed in 34% of patients and chronic graft-versus-host disease (cGVHD) in 38% of patients. Although not statistically significant, there was a higher incidence of overall aGVHD in Indian patients (73%) compared to Chinese and Malays (57% and 56% respectively). There was no significant difference in the incidence of aGVHD and cGVHD with the source of stem cells. Overall survival (OS) and disease free survival (DFS) was 50% and 60% at five years respectively. Multivariate analysis showed that patients transplanted in standard risk and those who had limited cGVHD had a significant better OS, (p = 0.05 and p = 0.05). Patients who had cGVHD and transplanted in standard risk had a better DFS, (p = 0.002 and p < 0.001). In summary, AHSCT in standard risk patients is associated with a better outcome than those transplanted in high risk and although not statistically significant, there is a higher incidence of aGVHD in Indian patients.

Topics: Adolescent; Adult; Disease-Free Survival; Female; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Malaysia; Male; Middle Aged; Retrospective Studies; Transplantation, Homologous

The sole BMT centre in Malaysia caters only for children. Since 1987, 89 transplants have been performed using reverse barrier nursing techniques. The overall survival rate is 73% with the majority of survivors leading normal lives. The early and late infection rates of 46% and 13%, respectively, are comparable to those of other centres. Although the early septicaemia rate is 36% the immediate mortality rate is < 10%. GVHD is less frequent and severe and the interstitial pneumonitis rate lower than that in the West. The average cost of US $8000 per transplant is much lower than the cost of a transplant performed overseas. Thus we believe that our paediatric BMT programme is simple and cost-effective.

Topics: Adolescent; Bone Marrow Transplantation; Child; Child, Preschool; Female; Graft vs Host Disease; Humans; Incidence; Infant; Malaysia; Male; National Health Programs