CASE REPORT Rare association of Hodgkin lymphoma, Graves’ disease and myasthenia gravis complicated by post-radiation neurofibrosarcoma: coincidence or genetic susceptibility? Zso ´fia Simon Æ Zsuzsa Ress Æ Jo ´zsef Toldi Æ Anita Trauninger Æ Zso ´fia Milte ´nyi Æ A ´ rpa ´d Ille ´s Received: 31 August 2008 / Revised: 16 February 2009 / Accepted: 18 February 2009 / Published online: 21 April 2009 Ó The Japanese Society of Hematology 2009 Abstract With Hodgkin lymphoma (HL), other (auto- immune) 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 immunosup- pressant drugs, thus plasmapheresis, intravenous immuno- globulin 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 impor- tance of the risk or/and response adapted therapy of HL. Keywords Hodgkin lymphoma Á Graves’ disease Á Myasthenia gravis Á HLA Á Malignant peripheral nerve sheath tumour Á Neurofibrosarcoma Á Radiation therapy Á Late complication 1 Introduction Hodgkin lymphoma (HL) is a relatively rare lymphopro- liferative disease in Hungary. Nowadays, it can be treated successfully and the majority of patients will get in com- plete remission permanently and will be cured [1, 2], nevertheless, the late side effects of treatment must be calculated with. Our experience and data published in the literature indicate that—even though not too often—other diseases may occur or combine with HL when it starts or relapses, such as autoimmune haemolytic anaemia, immune thrombocytopaenia (ITP), nephrosis syndrome, Guillain-Barre ´ syndrome, myasthenia gravis (MG) suba- cute cerebellar degeneration (SCD), cutaneous abnormali- ties [3–5]. They are caused by autoimmune processes, and these abnormalities disappear after successful treatment of HL [3, 4]. Diseases of the thyroid gland, mostly thyreoiditis and hypothyroidism, are common among HL patients who received radiation therapy (RT) in the neck region, while Graves’ disease (GD) is developed more rarely. The pathomechanism of the disease involves immunologic processes and the contribution of the damaging effect of RT can also be supposed [5–7]. RT is proved to increase the chance of second malignancies in HL too, especially breast and lung carcinomas, whereas the malignant peripheral nerve sheath tumour (MPNST) is an extremely Z. Simon (&) Á Z. Ress Á Z. Milte ´nyi Á A ´ . Ille ´s 3rd Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Mo ´ricz Zs. str. 22, 4004 Debrecen, Hungary e-mail: zsocogo@gmail.com J. Toldi Regional Transfusion Center of the NBTS, Szeged, Hungary A. Trauninger Department of Neurology, University of Pe ´cs, Pecs, Hungary 123 Int J Hematol (2009) 89:523–528 DOI 10.1007/s12185-009-0281-x