RESUMEN
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.
Asunto(s)
Hemofilia A , Sepsis , Humanos , Anciano , Anciano de 80 o más Años , Hemofilia A/epidemiología , Hemofilia A/diagnóstico , Factor VIII , Estudios de Cohortes , Hong Kong/epidemiología , Respuesta Patológica Completa , Sepsis/complicacionesAsunto(s)
Adenocarcinoma del Pulmón , Carbazoles , Eritrocitos Anormales , Neoplasias Pulmonares , Piperidinas , Talasemia alfa , Adenocarcinoma del Pulmón/sangre , Adenocarcinoma del Pulmón/tratamiento farmacológico , Carbazoles/administración & dosificación , Carbazoles/efectos adversos , Eritrocitos Anormales/metabolismo , Eritrocitos Anormales/patología , Humanos , Neoplasias Pulmonares/sangre , Neoplasias Pulmonares/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Piperidinas/administración & dosificación , Piperidinas/efectos adversos , Talasemia alfa/sangre , Talasemia alfa/tratamiento farmacológico , Talasemia alfa/patologíaRESUMEN
Amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis. It can cause progressive organ dysfunction and eventually death, mainly due to cardiac involvement. Amyloidosis may rarely present as extensive amorphous, purplish-blue deposits in marrow aspirate smears. Demonstration of congophilic property and apple-green birefringence under polarized light in aspirate smears can allow a rapid diagnosis of amyloidosis.
