Spontaneous bleeding in a patient with malignant lymphoma: A case of acquired hemophilia

Background: Acquired hemophilia is a rare condition which can be associated with lymphoproliferative disease. Case Report: Eleven yea rs after the dia gnosis of immunocytoma had been made, a 72-year-old man developed a high-ti ter factor VIII inhibitor. At this time, the lymphoma was without significa nt progress and there was no paraprotein in the serum. Partial thromboplast in time (PTT) was 83 a, factor-VIII clotting activity was <1%, and inhibito r level was 50.4 Bethesda units. The patient presented with spontaneous hem atomas in the skin and musculature of the extremities. Following combinatio n chemotherapy with cyclophosphamide, vincristine and prednisolone (COP), t here was a prompt disappearance of the inhibitor and normalization of coagu lation; however, the patient developed serious infectious complications. Wh en the inhibitor recurred he was treated with low-dose cyclophosphamide and prednisolone. This time there was a more delayed response, but the inhibit or disappeared again completely. Two months after cessation of therapy, the re was again relapse. Conclusion: Causal relationship between lymphoma and acquired hemophilia remains speculative. At least in some cases of factor V III inhibitors associated with malignant disease, immunosuppressive therapy may be sufficient to suppress the inhibitor.