HLA-A, -B and -DRB1 genotyping and haplotype frequencies of 3892 cord blood units in the Hong Kong Chinese Cord Blood Registry.

HLA-A, -B and -DRB1 gene and haplotype frequencies have been calculated from 3892 southern Chinese unrelated cord blood units in a Hong Kong Cord Blood Registry. This is the first large-scale paper to report the distribution of A-B-DRB1 alleles in Hong Kong Chinese Cord Blood Units. This information is important for estimating the optimal and economically cost-effective donor size and likelihood of obtaining appropriately matched cord blood units for Chinese patients awaiting haematopoietic stem cell transplantation. The data are available in the Allele Frequencies Net Database under the population name ''Hong Kong Chinese Cord Blood Registry'' and the identifier (AFND003358).

Stereotactic radiosurgery for metastatic spine tumors.

Spinal metastases invariably affect the majority of patients with cancer. Many will develop symptoms related to pain and disability from epidural spinal cord compression as well as pathologic fracture of the vertebrae. With the emergence of targeted systemic therapies and a better understanding of cancer biology, patients are living longer with bony metastases. This poses particular challenges, as palliation of pain and maintenance of local tumor control are paramount to quality of life and overall functional independence for these patients. Stereotactic radiosurgery (SRS) has emerged as a potent primary standalone and adjuvant treatment option for spinal metastases. To date, the primary indications for SRS include 1) upfront standalone treatment for painful bony metastases in the oligometastatic patient, 2) standalone or post-operative treatment following progression or recurrence of local disease despite previous conventional external beam radiation therapy (cEBRT), and 3) following surgery during which epidural disease is decompressed and the spine stabilized when indicated. SRS has demonstrated a significant advantage over cEBRT for tumors traditionally regarded as relatively radioresistant such as sarcoma, melanoma, renal cell carcinoma, non-small cell lung cancer and colon carcinoma.9 The radiobiological advantage of increased tumoricidal dose delivery and spinal cord dose sparing in SRS have made this a powerful treatment alternative to cEBRT particularly within the context of re-irradiation. Given the limitations of spinal cord dose constraints, surgery is still the first-line therapy in patients with high-grade epidural spinal cord compression (ESCC). Epidural compression can be treated with SRS, however this risks radiation-induced myelopathy and challenges the safety of effective dose delivery at the dural margin.11 With increasing dose, radiation-induced vertebral fracture is the most serious and prevalent side effect of SRS.53 An overview of SRS, including the most common indications, complications, and outcomes for spinal metastases are presented here.

A pathway to insulin independence in newborns and infants with diabetes.

Permanent neonatal diabetes was previously assumed to require insulin injection or infusion for life. Recently, permanent neonatal diabetes resulting from mutations in the two protein subunits of the adenosine triphosphate-sensitive potassium channel (Kir6.2 and SUR1) has proven to be successfully treatable with high doses of sulfonylureas rather than insulin. Many patients with these mutations first develop hyperglycemia in the nursery or intensive care unit. The awareness of the neonatolgist of this entity can have dramatic effects on the long-term care and quality of life of these patients and their families. In this study, we present the experience of our center, highlighting aspects relevant to neonatal diagnosis and treatment.

Unrelated umbilical cord blood transplantation in children: experience of the Hong Kong Red Cross Blood Transfusion Service.

OBJECTIVE: To review the outcome of unrelated umbilical cord blood transplantation in children using cord blood from the Hong Kong Red Cross Blood Transfusion Service. DESIGN: Retrospective study. PATIENTS: Records of eight patients who received unrelated umbilical cord blood transplants between 1999 and 2003 were reviewed. MAIN OUTCOME MEASURES: Engraftment of haematopoietic cells and graft-versus-host disease after transplantation. RESULTS: The median age of the patients was 4.9 years (range, 1.0-9.4 years). Five patients had acute leukaemia, one had non-Hodgkin's lymphoma, one had X-linked adrenoleukodystrophy, and one had mucolipidosis. The infused umbilical cord blood units contained a median of 6.7 x 10(7) /kg nucleated cells and 4.0 x 10(5) /kg CD34-positive cells. Neutrophil engraftment was achieved at a median of 13 days (range, 11-19 days) and, for seven patients, platelet engraftment was achieved at a median of 39 days (range, 24-98 days). Acute graft-versus-host disease occurred in all patients (grades I to III). One of the patients died because of encephalitis; of the other seven, five developed chronic graft-versus-host disease of the skin. At a median follow-up of 2 years, the four patients with leukaemia and the one with non-Hodgkin's lymphoma remained in continuous complete remission; the patient with adrenoleukodystrophy showed stabilisation of neurological condition. CONCLUSION: The Hong Kong Red Cross Blood Transfusion Service Cord Blood Bank stored cord blood units of good quality for transplantation, the outcome of which was comparable to that of bone marrow transplantation.

Estimation of bacterial risk in extending the shelf life of PLT concentrates from 5 to 7 days.

BACKGROUND: The use of bacterial culture to prevent bacterial contamination of blood components has renewed interest for extending the shelf life of PLT concentrates to 7 days after collection. STUDY DESIGN AND METHODS: This study was therefore conducted to determine the residual risk of bacterial contamination in PLT concentrates at the end of 5 and 7 days after collection in a center where all PLT concentrates are routinely screened by taking samples on Day 2 for culture. PLT units with no growth after 48 hours were sampled a second time on Day 5 or Day 7 after collection, followed by inoculation into aerobic culture bottles. The inoculated bottles were then monitored for up to 7 days at 35 degrees C in an automatic monitoring and detection system. RESULTS: During a 16-month study period, a total of 6020 PLT concentrates were tested 5 days (Group A, n=3010) and 7 days (Group B, n=3010) after collection. Four units in each group (0.133%) were found to be contaminated. In 6 units, bacteria were seen on direct Gram stain. In addition, 5 of the associated RBC units grew the same organisms on culture. The organisms include three coagulase-negative staphylococci and five Propionibacterium acnes. The positive rate of routine short-term bacterial culture was 0.035 percent during the same study period. CONCLUSION: Despite routine short-term bacterial culture, a significant risk of bacterial contamination remains at 5 and 7 days after collection. For now, the shelf life of PLT concentrates should remain 5 days.

Impact of donor arm skin disinfection on the bacterial contamination rate of platelet concentrates.

BACKGROUND AND OBJECTIVES: Despite improved methods for detecting bacterial contamination of blood products, bacterial sepsis remains a significant risk in blood transfusion. This study was undertaken to investigate whether adopting a different skin disinfection protocol could reduce the rate of bacterial contamination of platelet concentrates. MATERIALS AND METHODS: Two skin disinfection protocols were consecutively used in the routine blood collection setting during two 10-month periods: 0.5% cetrimide/0.05% chlorhexidine solution followed by 70% isopropyl alcohol (first 10-month time-period); and 10% povidone-iodine followed by 70% isopropyl alcohol (second 10-month time-period). The rates of bacterial contamination of platelet concentrates were monitored by using a surveillance programme described previously. RESULTS: The overall bacterial contamination rate in the first time-period was 0.072%. After introduction of the povidone-iodine and isopropyl alcohol protocol, the bacterial contamination rate decreased to 0.042% (relative risk reduction: -0.42; 95% confidence interval, -0.12 to -0.61, P= 0.009). There were no differences in the types of micro-organisms identified (P = 0.7). CONCLUSIONS: Skin disinfection by povidone-iodine and isopropyl alcohol is more effective than that by cetrimide/chorhexidine and isopropyl alcohol in reducing venepuncture-associated contamination of platelet concentrates by skin flora. Our data indicate that the disinfection protocol should be used on a routine basis and such implementation should translate into a significant improvement in blood safety to patients receiving platelet transfusion.

Report on anti-Dib encountered in two Hong Kong Chinese.

Two cases of anti-Dib, a rarely encountered antibody, were identified in serum samples referred by hospital blood banks during the past 13 months. Case 1 is a 41-year-old female who required blood for elective surgery. Case 2 is a premature infant suffering from mild neonatal jaundice on day 2 after birth. The anti-Dib in both cases exhibited marked dosage effect. The titer/score against Di(a+b+) and Di(a-b+) red blood cells (RBCs) in case 1 was 8/10 and 32/32, respectively, and in case 2, 4/18 and 32/46. The monocyte monolayer assay (MMA) also gave a similar pattern of results, being l5 percent and 100 percent reactive when tested with Di(a+b+) and Di(a-b+) RBCs in case 1, and 0.4 percent (within normal range) and l4.4 percent in case 2. The patient in case 1 underwent her operation without blood transfusion. The infant in case 2 was treated by phototherapy and subsequently recovered without the need for exchange transfusion.

Detection and clinical features of hepatitis C virus type 6 infections in blood donors from Hong Kong.

The genotype distribution of hepatitis C virus (HCV) was investigated in 212 viraemic blood donors from Hong Kong. A subset of the samples was investigated using three different genotyping assays to establish the accuracy of each in this population. These assays were restriction fragment length polymorphism (RFLP) of amplified 5' noncoding region (5'NCR) sequences, RFLP of the core region, and a serotyping assay using peptides from two antigenic regions of NS4. Genotypes detected in Hong Kong blood donors were 1a (6.2%), 1b (58.8%), 2a (1.4%), 2b (1.4%), 3a (1.9%), and 6a (27.0%). All genotyping assays produced concordant results. No evidence was obtained for the presence of type 6 group variants recently identified in Southeast Asia, other than type 6a. A serotyping assay based upon the detection of type-specific antibody to epitopes in NS4 produced similar results to the genotyping assays (98% concordance), but a reduced sensitivity (75%) compared with genotyping methods. Sequence variation in NS4 was not the cause of the reduced rate of detection of type 6 antibody in this population. Eighty-four percent donors infected with type 6a were male, compared to 75% donors infected with type 1b. The median alanine transaminase (ALT) level in type 6 infected donors was lower than in type 1b, (43.8 and 51.1 U/l, respectively) although these values were not statistically significant (P = 0.094). There was no significant difference between the ages of donors infected with types 1b and 6a. Risk factors for HCV infection in the blood donors included blood transfusion, intravenous drug abuse, and tattooing. A significantly greater number of donors infected with HCV-6a reported a history of drug abuse (66%) than donors infected with HCV-1b (7%).

First case of haemolytic disease of the newborn due to anti-Mur in Hong Kong.

The fifth child of a Hong Kong Chinese mother developed moderate jaundice, attributable to antibodies (anti-Mi) against antigenic determinants in GP.Mur (Miltenberger, class III) red cells. Both the father and the eldest sister were of the phenotype GP.Mur. Testing of maternal serum against a red cell panel including cells known to carry the antigenic determinants of some Miltenberger phenotypes revealed the presence of anti-Mur. This report documents the first case of haemolytic disease of the newborn (HDN) due to anti-Mur in Hong Kong.

A case of hydrops fetalis, probably due to antibodies directed against antigenic determinants of GP.Mur (Miltenberger class III) cells.

The GP.Mur (Miltenberger class III) phenotype was found to occur in about 6.3 percent of Hong Kong (HK) Chinese blood donors. The incidence of antibodies directed against antigenic determinants of GP.Mur cells (anti-Mi) among patients was 0.34 percent, similar to that in Taiwan Chinese. A case of hydrops fetalis probably attributable to maternal anti-Mi was encountered in an HK Chinese woman during her sixth pregnancy. The anti-Mi was potent (titer 512, score 99). It fixed complement and was a mixture of IgG1 and IgG3. Two biological assays, the monocyte monolayer assay and the chemiluminescence test, were strongly positive. The father was found to be heterozygous for the GP.Mur gene.

Penicillium marneffei infection: an underdiagnosed disease?

Penicillium marneffei is a rare human pathogen that often causes problems in clinical and histological diagnosis. A patient who presented with autoimmune haemolytic anaemia and hepatosplenomegaly, and was subsequently found to be suffering from disseminated Penicillium marneffei infection, is reported. The liver biopsy showed epithelioid cell granulomas only, and tuberculosis was initially considered the most likely diagnosis. The correct diagnosis became evident in a subsequent colonic biopsy, which showed extensive infiltration by penicillium-laden macrophages.