A synopsis of current haemophilia care in Hong Kong

Objective To provide a synopsis of current haemophilia care in Hong Kong. Design Retrospective survey. Setting All haematology units of the Hospital Authority in Hong Kong. Patients All patients with haemophilia A and haemophilia B. Results To date, there were 222 mild-to-severe haemophilia patients (192 type A, 30 type B) under regular public care in Hong Kong (43% were considered severe, 33% moderate, and 24% mild), which gave a crude prevalence of 6.8/100 000 male inhabitants. A total of 12.8 million units of Factor VIII and 3 million units of Factor IX were prescribed annually. This amounts to 1.83 units of FVIII per capita of the population, which is comparable to that of other developed countries. Leading causes of mortality were human immunodefciency virus-related complications (10 cases) and cerebral bleeding (2 cases). The life expectancy of patients with severe haemophilia in Hong Kong is improving; currently the oldest patient is 60 years old. Such improved survival may be due to enhanced factor availability, prompt treatment of bleeding episodes at home, safer factor products, and better antiviral treatment. Primary prophylaxis is the accepted standard of care for severe and moderate cases, and "Factor First" has become hospital policy. However, 12 patients continue to present treatment challenges, due to the documented presence of factor inhibitors. In all, 28,100, and 14 cases respectively were positive for human immunodefciency virus, hepatitis C virus, and hepatitis B virus; the youngest patients with the corresponding infections being 28,13, and 22 years old. Comprehensive care with dedicated physiotherapy, surgical support, and radionucleotide synovectomy may reduce morbidity further. Conclusion A multidisciplinary approach can further improve the future care for haemophilia patients in Hong Kong.published_or_final_versio

A synopsis of current care of thalassaemia major patients in Hong Kong

OBJECTIVE: To provide a synopsis of current thalassaemia major patient care in Hong Kong. DESIGN: Retrospective study. SETTING: All haematology units of the Hospital Authority in Hong Kong. PATIENTS: All patients with thalassaemia major with regular transfusion. RESULTS: To date, there were 363 thalassaemia major patients under the care of the Hospital Authority. Prenatal diagnosis has helped to reduce the number of indigenous new cases, but in recent years immigrant cases are appearing. The patients have a mean age of 23 (range, 1-52) years, and 78% of them are adults. In 2009, they received 18 782 units of blood. This accounted for 9.5% of all blood consumption from the Hong Kong Red Cross. In the past, cardiac iron overload was the major cause of death (65%) and few patients survived beyond the age of 45 years. The availability of cardiac iron assessment by magnetic resonance imaging (T2 MRI) to direct the use of oral deferiprone chelation has reduced the prevalence of heart failure and cardiac haemosiderosis, which should reduce mortality and improve life expectancy. CONCLUSION: The future for thalassaemia care in Hong Kong is bright. With better transfusion and chelation, it should be possible to avoid growth and endocrine deficiencies in younger patients.published_or_final_versio

HLA alleles associated with asparaginase hypersensitivity in Chinese children

Asparaginase is an important drug to treat childhood haematological malignancies. Data on the association between human leukocyte antigens (HLA) and asparaginase hypersensitivity among Chinese are lacking. We conducted a retrospective study to identify HLA alleles associated with asparaginase hypersensitivity among Chinese children with acute lymphoblastic leukaemia (ALL), mixed phenotype leukaemia and non-Hodgkin lymphoma (NHL), who received asparaginases with HLA typing performed between 2009 and 2019. 107 Chinese patients were analysed. 66.3% (71/107) developed hypersensitivity to at least one of the asparaginases. HLA-B*46:01 (OR 3.8, 95% CI 1.4-10.1, p < 0.01) and DRB1*09:01 (OR 4.3, 95% CI 1.6-11.4, p < 0.01) were significantly associated with L-asparaginase hypersensitivities, which remained significant after adjustment for age, gender and B cell ALL [HLA-B*46:01 (adjusted OR 3.5, 95% 1.3-10.5, p = 0.02) and DRB1*09:01 (OR 4.4, 95% CI 1.6-13.3, p < 0.01)]

Review on the management and outcome of childhood cerebral or cerebellar low grade glioma

Abstract Topic: Disease Oroented: no. OP-276INTRODUCTION: Astrocytoma is the commonest type of brain tumors in childhood and low grade (WHO Gr I & II) accounted for almost half of them. Despite having good prognosis, the management approach remains quite variable in different parts of the world. AIM: We reviewed our experience on this group of patients. MATERIALS AND METHODS: This is a retrospective review of a population based cohort from Jan-1999 to Dec-2014. The patients underwent treatment in 5 local hospitals which captured almost all children with cancers locally. The patients’ data were recorded prospectively by 2 full time data managers. The data was verified annually with the respective centers and HK Cancer registry. RESULTS: Within this 16 yrs period, there were 157 children with astrocytoma diagnosed. They were distributed as following: Cerebral & Cerebellar: n = 107 (Gr I n = 44, Gr II n = 21, Gr III n = 14, Gr IV n = 28); Brainstem: n = 50; Spinal: n = 9 (Gr I n = 4, Gr II n = 1, Gr III n = 1, Gr IV n = 4); Other types of glioma: n = 20 (Oligodendroglioma n = 10, PXA n = 2, Ganglioglioma n = 7, SEGA n = 1). Excluding the spinal astrocytoma and other types of glioma, we identifi ed 65 patients with cerebral or cerebellar low grade astrocytoma as mentioned above. Their median age was 7.95yrs (range 0.15 to 17.81yrs) and M:F = 35:30. Cerebellum is the most common location (n = 28) followed by suprasellar region (n = 10). Surgery was the main form of treatment (n = 57) and 18 received additional low intensity chemotherapy. The choice of diff erent regimens depended on diff erent era, including carboplatin + vincristine, temozolomide alone, vinblastine alone, TPGV, PCV, etc. Patients often received more than one course of chemotherapy. Two patients underwent RT in the early 2000 era. Only 3 patients died of disease progression (all WHO Gr I). The 5-year event free survival was 93.3 % and 100 % for Gr I & Gr II patients respectively. ONCLUSION: Childhood low grade astrocytoma has relatively good prognosis even if unresectable. Low intensive chemotherapy could suppress the progression of a significant proportion of these low grade tumors and achieve long term survival. Even if it recurred, we could utilize chemotherapy again and avoid using RT on this group of patients

Treatment Outcome of Metastatic Neuroblastoma with Antiganglioside 2 Monoclonal Antibody (3F8): the Hong Kong Experience

SIOP Awards SessionPurpose: The prognosis of metastatic neuroblastoma remains to be poor despiteintensive chemotherapy and radiation therapy. Immunotherapy with monoclonalantibody appears to provide an additional armamentarium to the current treatmentstrategy. We evaluated our experience in using this approach.Method: This is a prospective study from Jan 1996 to Dec 2007 and the results werecompared to our historical control from 1990 when a common data registry wasstarted. Uniform treatment protocol was adopted by 5 public hospitals since 1996. Ourtreatment was based on a modified N6 & N7 protocol adopted from MSKCC whichincluded intensive chemotherapyþautologous BMT þ/ immunotherapy withmurine monoclonal anti-ganglioside-2 antibody (3F8, anti-GD2 provided by MSKCCafter 1999) for patients with stage 4 neuroblastoma. The analysis of outcome was byKaplan-Meier analysis.Results: Within these 13 years, 61 children (all >12 months) with metastaticneuroblastoma were diagnosed. The 5 years PFS were 11.8% and 49.1% after treatedwith chemotherapy þ/ auto-PBSCT (n¼34) and chemotherapyþ auto-BMTþ3F8(n¼27) respectively (p¼0.009). Their median survival were 1.8 yrs vs. 4.9 yrsrespectively (p¼0.015). The most frequent encountered side effect of 3F8 wastransient but severe pain and allergic reaction. The median follow-up period is3.8 years and the minimal follow-up period is two year.Conclusion: Patients with stage 4 neuroblastoma treated with 3F8 antibody appearedto have a higher and longer survival as compared to the historical control. Other frompain which could readily be controlled with analgesic, the side effects were acceptablein our cohort

A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong

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Cardiac Magnetic Resonance Assessment of myocardial iron load and fibrosis in adult survivors of childhood leukaemias

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Clinical Presentations and Outcome of Hospitalised Paediatric Oncology Patients with Laboratory-confirmed Pandemic H1N1 Influenza Infection in Hong Kong MMK SHING Pandemic H1N1 Infection in Paediatric oncology Patients 199

Abstract Objective: The clinical course of hospitalised paediatric oncology patients and haematopoietic stem cell transplant (HSCT) recipients with laboratory-confirmed pandemic H1N1 influenza infection were studied. Methods: Data from oncology patients and HSCT recipients with hospitalised laboratory-confirmed pandemic H1N1 influenza infection in Hong Kong were collected. Parameters on initial presentations, clinical course, treatment regimens and outcome were studied. Results: Sixteen patients were studied, the median age was 12.4 years. Fever (100%), cough (75.0%), runny nose (56.3%) and sore throat (50.0%) were the most common presenting symptoms. Antiviral therapy was started at median 1 day after onset of fever. HSCT recipients were more common to require a repeated or prolonged course of antiviral therapy due to persistent respiratory symptoms. Our cohort recovered without severe complications. Conclusion: Fever, cough, runny nose and sore throat were the most common presenting symptoms. HSCT recipients were more likely to develop persistent or recurrent respiratory symptoms and required repeated course of antiviral therapy. The uncomplicated course of pandemic H1N1 infection of paediatric oncology and HSCT recipients might be related to the early initiation of antiviral therapy