1. BACKGROUND Sezary syndrome and mycosis fungoides are cutaneous T- cell lymphomas characterized by the epidermotropism of tumor cells. 1 Their primary manifestation is reddened, itchy, peeling, and painful skin all over the body. There may also be patches, plaques, or tumors on the skin. 1 Extracutaneous spread is not uncommon. Atypical cancerous T-cells (Sezary cells) are found in the peripheral blood. 2 Lymphadenopathy and hepatosplenomegaly could be seen. But neuropathic disease is very rare. In search of English literature there were three cases of polyneuropathy with cutaneous T-cell lymphoma and Sezary Syndrome reported. 3-5 Herein a case of Sezary Syndrome with motor axonal neuropathy is presented. PERIPHERAL NEUROPATHY IN SEZARY SYNDROME: COINCIDENCE OR A PART OF THE SYNDROME? Yesim Sucullu Karadag, Neşe Subutay Oztekin, Aydın Gulunay, Fikri Ak Numune Education and Research Hospital Neurology Clinic, Ankara, Turkey P30-03 Case Report A 48 year old male patient was admitted with complaints of diffuse skin dryness, fatique, numbness at hands and edema at ankles for 6 months. He reported cramps and fasciculations occurring at nights and weakness of the arms after lifting heavy packages. In another hospital, Gabapentin was recommended but his complaints had not resolved. Unlikely edema had occurred in his hands and legs and right inguinal region. In dermatological examination a diffuse dryness with widespreaed squams was found at skin with hair, trunk, arms and thighs. (Figure 1- 2). There were partially erythematous changes at arms and thighs. Inguinal lympadenopathy was established. Neurologic examination revealed hypoactive deep tendon reflexes in both lower extremities and glove-socks wise hypoestesia. In laboratory analysis, white blood cell count was 43.600/mm 3, 50- 55% of them were eosinophils. Flow cytometry revealed a Sezary cell count of 3200/μL, a CD4/CD8 ratio greater than 22,5, and less than 7% Tcells that expressed CD7. The same clonal amplification of T cells was demonstrated by T-cell receptor gene analysis in skin, blood, and lymph nodes. The diagnosis of SS was established. 6 There was multiple axillary and inguinal lymphadenopathies with maximal size of 25x17 mm in computerized tomography. As he had skin eruption, dryness and diffuse sclerodermoid appearance on his upper extremities, thin white squams adherent especially in the occipital region of the hairy skin muscle and skin biopsy were performed. Patients’ punch skin and lymph node biopsies were consistent with Sezary Syndrome showing diffuse CD4 staining. Muscle biopsy showed chronic inflammatory changes. CONCLUSION Patients with cancer may develop signs and symptoms of peripheral nerve dysfunction. Polyneuropathy could be related to chemotherapy, occasionally to leptomeningeal disease, and rarely due to direct invasion of the nerve. 8 In some disorders, neuronal antigens expressed by the tumor stimulate an immune response characterized by T cells, antibodies or both, that not only attack the tumor but also the neural tissue. 9 Although these scenerios could be seen in lymphoma nervous system involvement is not reported in reviews of MF and SS. 2-,10 So far there was a case reported in 1978 by Bargman and Coupe relating the co-occurrence of Sezary Syndrome and peripheral neuropathy. 5 The other case of SS with neurolymphomatosis was reported and proved by histologic examination by Bezier et al. In this case report histologic examination of a muscle and nerve biopsy specimen showed neurogenic muscle atrophy and an axonal neuropathy secondary to an epineurial and endoneurial infiltration by Sezary cells. 3 Neurolymphomatosis is a rare neurologic manifestation of systemic lymphomas. Although an extracutaneous spread is not uncommon in advanced stages of MF and SS, neurologic complications are rare and generally result from leptomeningeal or central nervous system involvement. Our patient had an axonal peripheral neuropathy without central nervous system involvement. Neuropathic symptoms started prior to his medications and so this leaves only his primary disease. This unusual and very rare case emphasizes the importance of bearing polyneuropathy in mind while dealing with patients with Sezary syndrome although it is difficult to treat and suggests the possible role of neurotropism of malignant cells in Sezary syndrome. His electromyography revealed motor axonal neuropathy. Other etiologies for motor aksonal neuropathy such as diabetes mellitus, intoxication, hereditary, chronic renal failure, alcoholism were all excluded. Interferon alpha 2-a treatment was started. PUVA therapy was used for his skin lesions. Photopheresis was done once a month. Pregabalin was started for his parestezias and pain and his symptoms regressed. Although we could not perform nerve biopsy, no reponse to gabapentin, and the initiation time of the symptoms such as paresthesias, support the diagnosis of polyneuropathy due to Sezary syndrome. Nowadays the patient is on a preparative treatment for stem cell transplantation. REFERENCES Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and S é zary syndrome. Lancet. 2008 Mar 15;371(9616):945-57.Hwang STJanik JEJaffe ESWilson WHLancet. Kubica AW, Davis MD, Weaver AL, Killian JM, Pittelkow MR. S é zary syndrome: A study of 176 patients at Mayo Clinic. J Am Acad Dermatol. 2012 May 26.S é zary syndrome: A study of 176 patients at Mayo Clinic. Bezier M, Reguia ï Z, Delaby P et al.. Neurolymphomatosis Associated With Se´zary Syndrome Arch Dermatol. 2009;145(3):294-296 Atiq OT, DeAngelis LM, Rosenblum M, Portlock CS. Cutaneous T-cell lymphoma presenting with diffuse lymphomatous infiltration of the peripheral nerves: response to combination chemotherapy. Am J Clin Oncol. 1992 Jun;15(3):212-5.Cutaneous T-cell lymphoma presenting with diffuse lymphomatous infiltration of the peripheral nerves: response to combination chemotherapy. Bargman H, Coupe RL. S é zary's syndrome: report of a case showing peripheral neuropathy and bone marrow fibrosis. Arch Dermatol. 1978 Sep;114(9):1360-2.S é zary's syndrome: report of a case showing peripheral neuropathy and bone marrow fibrosis. Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. (EORTC). Blood 2007;110:1713-22. John D England, Arthur K AsburyPeripheral neuropathy. Lancet 2004; 363: 2151 – 61 Viala K, Be´hin A, Maisonobe T,Neuropathy in lymphoma: a relationship between the pattern of neuropathy, type of lymphoma and prognosis? J Neurol Neurosurg Psychiatry 2008;79:778 – 782. Rudnicki, SA; Dalmau J. Paraneoplastic syndromes of the peripheral nerves Current Opinion in Neurology.2005; 18(5):598-603,Paraneoplastic syndromes of the peripheral nerves Sam T Hwang, John E Janik, Elaine S Jaff e, Wyndham H Wilson Mycosis fungoides and S é zary syndrome Lancet 2008; 371: 945 – 57 Figure 2. Figure 1.