Rare association of Hodgkin lymphoma, Graves’ disease and myasthenia gravis complicated by post-radiation neurofibrosarcoma: coincidence or genetic susceptibility?

Abstract

With Hodgkin lymphoma (HL), other (autoimmune) diseases may occasionally occur or associate, whereas as a late treatment-complication, second tumour may develop. In our patient HL was diagnosed in 1996 and consequently received COPP/ABV and mantle irradiation. Due to the residual mediastinal tumour CRu was declared but later on no progression/relapse could be proved by PET. In 2000 Graves's disease, in 2001 myasthenia gravis was diagnosed, which showed resistance for immunosuppressant drugs, thus plasmapheresis, intravenous immunoglobulin treatments were applied. In 2005, the residual mediastinal tumour started progressive growth, which leads to thoracotomy in which the tumour was removed, it was malignant peripheral nerve sheath tumour. The disease showed progression despite the chemotherapy applied and the patient died in 2007 due to respiratory failure. Not even the postmortem histopathologic examination revealed the relapse of HL. Association of Hodgkin lymphoma, and two antibody-mediated autoimmune diseases, Graves' disease and myasthenia gravis, is rare and has not yet been reported in the literature. The etiologic role of genetic predisposition and immune regulatory disorder must definitely be thought of, as the possibility of mere coincidence is extremely small. Malignant peripheral nerve sheath tumour is a rare complication of irradiation, which underlines the importance of the risk or/and response adapted therapy of HL.