Characteristics and outcome of a territory-wide cohort study of patients with acquired hemophilia A in Hong Kong.

INTRODUCTION: Acquired hemophilia A (AHA) is a rare bleeding disorder with destruction of factor VIII by autoantibodies. Comprehensive data for Chinese patients are lacking. Predictors of hospital stay have not been investigated. METHODS: A territory-wide review of patients diagnosed with AHA from January 1, 2012, to December 31, 2021 was performed by retrieving patients' information from an electronic database system in Hong Kong. RESULTS: Overall, 165 patients were included in this 10-year study, and the estimated incidence was 2.4 per million/year, which was higher than those reported from Caucasian cohorts. The median age of diagnosis was 80 years old. Patients had a long hospital stay (median: 25 days) and high mortality (55.2 %). The majority of deaths were caused by immunosuppression-related sepsis (49.5 %). Age was an independent predictor of overall survival (Hazard ratio: 1.065, 95 % CI: 1.037-1.093, p < 0.001), complete remission (CR) status (odd ratios (OR): 0.948, 95 % CI: 0.921-0.976, p < 0.001) and time to achieve CR (OR: 1.043, 95 % CI: 1.019-1.067, p < 0.001). Higher hemoglobin level on presentation was associated with shorter time to achieve CR (OR: 0.888, 95 % CI: 0.795-0.993, p = 0.037). Factor VIII level < 1 % normal, high inhibitor titer and intensive immunosuppressive regimen predicted long hospital stay. CONCLUSION: We presented comprehensive data of Chinese patients with AHA which comprised predominantly frail elderly who required long hospital stay and had high sepsis-related mortality. This posed challenges in managing AHA in such patients. Individualized immunosuppressive therapy is needed to balance the benefits and risk of septic complications.

Breast Conserving Surgery for BRCA Mutation Carriers-A Systematic Review.

Similar to mastectomy, breast conserving surgery (BCS) is currently the reference standard of surgical treatment of sporadic breast cancer in patients. However, its oncologic safety for BRCA mutation carriers has remained controversial. Thus, we conducted a systematic review to critically evaluate the best evidence from reported studies. A comprehensive search was performed of the Medline, EMBASE, CINAHL, and Cochrane databases using a predefined strategy. The retrieved studies were independently screened and rated for relevance. Data were extracted for qualitative synthesis in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol for systematic reviews. No randomized controlled trial has directly compared BCS and mastectomy for BRCA mutation carriers. Of the 18 studies included in our review, the pooled analysis of overall survival at 5, 10, and 15 years were comparable between BCS and mastectomy (88.7%, 89.0% and 83.6% with BCS and 83%, 86.0%, and 83.2% with mastectomy, respectively). However, the pooled ipsilateral breast cancer recurrence rates at 5, 10, and 15 years were higher in the BCS group (8.2%, 15.5%, and 23%, respectively) than in the mastectomy group (3.4%, 4.9%, and 6.4%, respectively). BCS was associated with a greater rate of ipsilateral breast cancer recurrence in BRCA mutation carriers. However, it was not associated with adverse short- and long-term survival outcomes. BCS should be offered as an option to BRCA mutation carriers with proper preoperative counseling.