Acquired Factor VIII Inhibitor in a Patient with Tuberculosis

Dr. M.H. Aurousseau, Laboratoire Central d’Hématologie, Hôpital Jean Verdier, Avenue du 14 Juillet, F-93143 Bondy Cedex (France) Dear Sir, Acquired inhibitors against factor VIIIC in patients without hemophilia are the most common type of acquired specific antibodies but still a rare occurrence. We describe a 63-year-old man who presented with an idiopathic acquired hemophilia, revealed by a spontaneous hematoma in the thigh. No particular etiology was evidenced. Factor VΠIC was less than 1% with an inhibitor of 6 Bethesda Units. He was first treated with prednisone (1 mg/kg) in combination with highdose intravenous γ-globulins (IVIg): 400 mg/kg for 5 days. The inhibitor disappeared, then reappeared when corticosteroids were tapered with bleeding diathesis and a weak inhibitor against porcine factor. Several hemorrhagic events were treated by prothrombin complex concentrate (CPP) and activated PPC. After five cycles of cy-clophosphamide, the inhibitor seems to be eradicated and factor VIIIC increased to 41%. At that time, nodular opacity of the lower lobe was observed in the right lung. Neoplasia was suspected on biopsy, and lobectomy was performed after IVIg and activated PPC infusion. Microscopic examination revealed typical tubercular lesions and the patient was then treated with antituberculous agents. Thirty-three months after surgery, factor VIIIC inhibitor has not reappeared and factor VIIIC is completely normalized at 160%. Acquired inhibitors to factor VIIIC in non-hemophilic patients are rare and mostly encountered in patients over 50 years of age. As reported by Green and Lechner [1] in 1981, they may develop in association with autoimmune disorders, pregnancy, malignancy or drugs, but in 46% of patients there is no detectable underlying disorder. Our patient suffered from pulmonary tuberculosis: this etiology was not reported among the various disorders listed in this survey of 215 non-hemophilic patients. However, acquired factor VIIIC inhibitor has been described associated with pulmonary disease: lung abscess [2], bronchogenic and lung carcinoma [3, 4]. Furthermore, tuberculosis was occasionally described in association with an inhibitor against human factor V [5] protein which shares different homologous sequences with factor VΠIC. Cyclophosphamide, most of the time combined with prednisone, has been shown to be effective for long-term immunosuppression [6], and the inhibitor seems to disappear in our patient before antitu-