Development of Factor Vlll:C Inhibitors Following Vaccination

G.M. Ferri, MD, Department of’Medicina Clinica’, V.le dell’Università 37, I-00185 Rome (Italy) Acquired inhibitors against factor VIIL·C (FVIIL·C) in nonhemophilic patients are associated with various conditions, including autoimmune diseases (mainly rheumatoid arthritis and systemic lupus erythematosus), malignancies, the postpartum state, drug reactions, and dermatological disorders [1-4]. One case of circulating inhibitor associated with viral infection has also been described [5]. In this report, we describe the case of a 57-year-old male, diagnosed with lichenoid dermatosis, who developed severe hemorrhagic manifestations due to the appearance of inhibitors against FVIIL·C following vaccination with BCG and a pool of various strains of live attenuated corynebacteria. A 57-year-old male was admitted to our hospital in August 1989 with severe fatigue, bleeding from the gums, macroscopic hematuria, and spontaneous leg and forearm pain. He had no family or past history of a tendency to bleed. In September 1988, a lichenoid dermatosis of the scalp was diagnosed. Because of worsening of the skin lesions, vaccine therapy with BCG and a pool of various strains of live attenuated corynebacteria was advised, and was started in June 1989 in a private clinic, after the patient had given his informed consent. This therapeutic approach was adopted on the basis of preliminary unpublished results showing the efficacy of nonspecific immu-notherapy in lichenoid dermatosis. The vaccine was administered monthly by subcutaneous injection. Following the second booster, the hemorrhagic manifestations occurred. On examination, the patient appeared in poor general condition. Swelling of both calves and of the right forearm were present, due to the presence of hematomas. The liver and spleen were palpable 2 and 1 cm below the costal margin, respectively. Laboratory investigations revealed Hb 12.7 g/dl, WBC 5.2 × 109/1 (72% neutrophils, 26% lymphocytes, 1 % monocytes, and 1 % eosinophils), platelets 229 × 109/1 and ESR 80 mm in 1 h. Macroscopic hematuria was present. Liver enzymes, serum creatinine, and blood urea nitrogen were normal. Tests for VDRL, rheumatoid factors and antinuclear antibodies were negative. The prothrombin time (PT), the activated partial thromboplastin time (aPTT), and the quantification of ñbrinogen