Razi Journal of Medical Sciences

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We reported a case of primary centeral nervous system lymphoma(PCNSL) following Behcet’s syndrome. A 24-years-old man with a past history of muco-cutaneous lesions including oral and genital aphthosis, facial acen, feet ulcer, and anterior uveitis since May 1999 visited the Behcet’s clinic and full work-up was performed. During this time. Patient showed abnormal behavior, aggressiveness and agitation. Brain CT scanning and laboratory data were compatible with Behcet’s syndrome and ite CNS vasculitis. Treatment started and continued in several stages with colchicine, prednisolone, chlorambucil and pentoxyphiline with resultant partial response. On December 2001, patient came in emergency ward with symptoms and signs of increased intracranial pressure. Brain CT scanning was done and an emergency craniotomy was performed that revealed a posterior-fossa tumor. Tumor resection done and pathological examination showed non-Hodgkin’s lymphoma (NHL), diffuse large cell type. Staging procedures was performed and showed no sign of other organ involvement. So, with impression of PCNSL, patient received radiation therapy and chemotherapy with good response. He is now under supervision of out-patient clinic. This is the second case of PCNSL associated with Behcet’s syndrome reported on the basis of literature review at the end of 2001. It seems that occurrence of NHL in Behcet’s syndrome is related to immunosuppressive state induced by therapeutic agents such as colchicine, etc. or the disease as a premalignant state. Therefore, Behcet’s patients should be under special supervision in point of view of developing malignancies.

Keywords: 1) Primary central nervous system lymphoma 2) Non-Hodgkin’s lymphoma 3) Behcet’s syndrome

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