Tuen Mun Hospital

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Data among Chinese patients is sparse. This multicenter retrospective study aims to assess the presentation, treatment strategies, and outcomes of EGPA in Hong Kong. Methods: Patients with newly diagnosed EGPA who presented to eight participating hospitals in Hong Kong between January 1, 2013 and December 31, 2023 were identified. Clinical history and laboratory parameters were collected and analyzed. Outcomes, including mortality, relapse, and complications from disease and treatment, were assessed. Results: Seventy-six patients were identified. The annual incidence of EGPA was estimated at 1.9 per million population. Regarding the presentation, neurological symptoms (71.1%) were most frequently observed, followed by cutaneous (54%) and pulmonary symptoms (52.6%). The presence of anti-myeloperoxidase antibody (anti-MPO) correlated with a higher Birmingham Vasculitis Activity Score (BVAS) on presentation, more renal involvement, and absence of asthma. While anti-PR3 was associated with cardiovascular manifestations. Treatment mainly involved corticosteroids and cyclophosphamide as induction and azathioprine as maintenance. Biologics were used for induction in 18.4% of cases and for maintenance in 7.9%. Infective complications occurred in 29% of patients; a starting prednisolone dose [Formula: see text] 40 mg daily and a history of diabetes mellitus were identified as risk factors. Relapses occurred in 26.3% of subjects; the mean time to relapse was 39.5 months. Multivariate survival analysis revealed that a history of smoking [Formula: see text] 1 year (HR 20.63, 95% CI 2.76–154.37, [Formula: see text] = 0.03), absence of asthma (HR 0.13, 95% CI 0.02–0.96, [Formula: see text] = 0.045), and absence of neurological involvement on presentation (HR 0.06, 95% CI 0.01–0.45, [Formula: see text] = 0.006) were associated with mortality. Conclusion: This study underscored the heterogeneity of EGPA in the Chinese population and supports categorization based on anti-MPO status. Smoking history and absence of asthma or neurological symptoms were associated with mortality. The findings aim to inform clinical practice and direct future research efforts in managing this complex disease.

von Hippel-Lindau (VHL) syndrome is a rare autosomal dominant genetic disorder that typically leads to the development of multiple tumours in various organs. This study describes the lifetime journey of VHL patients in terms of their hospitalisation, surgery, and functional impairment, and aims to examine the local presentation patterns, treatment courses, and clinical outcomes associated with the condition. Thirty-two patients with VHL syndrome (mean age=27.9 ± 12.6 years) were retrospectively identified from five local public hospitals managed between 1 January 1993 and 30 September 2024, with a follow-up duration of 18.0 ± 10.8 years. Patient demographics, disease presentation, length of hospital stay, and treatments received were recorded and analysed. Over a total of 575.9 person-years, 17 patients (53.1%) developed renal tumours and 10 (31.3%) underwent partial or radical nephrectomy. Twenty-four patients (75.0%) underwent central nervous system (CNS) surgery for haemangioma. Eleven patients (34.4%) had phaeochromocytoma, and eight (25.0%) underwent adrenalectomy. Nine patients (28.1%) had retinal haemangioma. During the study period, 368 emergency department visits, 1209 inpatient admissions, 192 intensive care unit days, and 5635 hospitalisation days were recorded. In total, 116 surgeries were performed involving the kidneys (n=17), pancreas (n=6), adrenal glands (n=10), and CNS (n=83). Six patients required dialysis; 4373 dialysis sessions were performed. Fifteen patients died. Among the nine who died of VHL syndrome, eight had developed cerebral haemangioblastoma, three had phaeochromocytoma, and four had renal tumours. Patients with VHL syndrome often experience early-onset and recurrent diseases affecting multiple organ systems, leading to substantial morbidity and mortality. A multidisciplinary approach, along with the introduction of novel treatments, may improve disease control and clinical outcomes.

Enchondroma is the most common benign bone tumor of the hand. It has a predilection for the ulnar-sided tubular bones of the hand and occurs most common in the proximal phalanx, less commonly in the middle phalanx and metacarpals, and rarely in the distal phalanx. The purpose of this Technical Note is to report the technical details of endoscopic curettage and bone grafting of enchondroma of the metacarpal head. Endoscopic curettage of enchondroma has been reported to ensure complete removal of tumor tissue and reduce the risk of iatrogenic fracture.

Background:The pivotal trial on venetoclax and hypomethylating agents in unfit elderly acute myeloid leukaemia (AML) has got FDA approval. However, Asian patients were under-represented and showed no survival advantage.Objective:We aimed to compare overall survival and healthcare resource utilisation in elderly patients newly diagnosed with AML, receiving venetoclax with hypomethylating agents versus hypomethylating agents alone.Design:Target trial emulation.Methods:Propensity score matching balanced the baseline characteristics. Kaplan-Meier curve and Cox regression compared overall survival. Negative binomial regression assessed healthcare resource utilisation with time offset.Results:Venetoclax plus hypomethylating agents treatment conferred superior overall survival in patients with AML ⩾60 years old and significantly reduced transfusion requirement compared to those receiving hypomethylating agents alone. The difference was particularly prominent among patients ⩾75 years old.Conclusion:Compared to hypomethylating agent alone, venetoclax plus hypomethylating agent benefits elderly patients with AML on overall survival and healthcare resource utilisation.

The pathophysiology of systemic lupus erythematosus (SLE) is complex and involves most cell types of the innate and adaptive immune system. Impaired clearance of apoptotic bodies and self-antigens, dysregulated cytokine network and aberrated functions of the immune cells lead to overproduction of autoantibodies, activation of complements, immune complex deposition and tissue injury. Novel biological and newer generation immunosuppressive agents have been developed to target the B cells, T cells, T/B cell interaction, plasmacytoid dendritic cells and the cytokines. With the advances in the knowledge about the intracellular pathways, small molecules that inhibit the downstream signal transduction from surface receptors and intracellular protein degradation by the ubiquitin-proteasome system are being developed in the pipeline. This article summarizes the evidence of various immunotargets for the treatment of SLE. These novel agents target specific cellular mechanisms, and further works are necessary to stratify patients according to biomarkers to receive individualized therapies that could help maximize the clinical response. With the availability of more therapeutic choices, a combination approach to achieve synergistic effects while reducing adverse events by dosage reduction of individual drugs is being explored for SLE patients at risk of disease progression or refractory to conventional therapies.