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.

Diagnostic and treatment hurdles in plasma cell myeloma with t(11;14) translocation: A case report.

t(11;14) translocation is one of the most common chromosomal abnormalities in plasma cell myeloma. The present case report presented a case of plasma cell myeloma with t(11;14) translocation, in which the plasma cells were small lymphoplasmacytoids in morphology with positive cluster of differentiation-20 and Cyclin D1 expression. These results led to initial diagnostic difficulties. The patient was refractory to bortezomib-based therapy, and responsive to vincristine, doxorubicin and dexamethasone. However, the prognostic value of t(11;14) in plasma cell myeloma remains to be determined. With recent advances in treatment options, physicians should be aware of the clinical and pathological characteristics of this translocation in plasma cell myeloma.