RESUMO
The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.
Assuntos
Hemofilia A , Idoso , Autoanticorpos , Fator VIII , Feminino , Hemofilia A/diagnóstico , Hemofilia A/tratamento farmacológico , Hemofilia A/epidemiologia , Humanos , Masculino , Sistema de Registros , Estudos RetrospectivosRESUMO
A 21-year-old man presented to hospital with a two-month history of productive cough with no other symptoms. Radiology revealed a cavitating lesion in the left upper lobe for which a variety of diagnoses were considered. A biopsy revealed primary pulmonary Hodgkin's lymphoma. Primary pulmonary Hodgkin's lymphoma is an uncommon initial presentation; lung lesions usually occur later in the course of the disease. Following diagnosis, the patient began chemotherapy and full remission was achieved.