لنفوم اوليه سيتسم عصبي مركزي به دنبال سندرم بهجت : گزارش يك مورد

PRIMARY CENTERAL NERVOUS SYSTEM LYMPHOMA FOLLOWING BEHCETʹS SYNDROME(CASE REPORT)

We reported a case of primary centeral nervous system lymphoma(PCNSL) following Behcetʹs 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 Behcetʹs 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 Behcetʹs 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-Hodgkinʹs 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 Behcetʹs syndrome reported on the basis of literature review at the end of 2001. It seems that occurrence of NHL in Behcetʹs syndrome is related to immunosuppressive state induced by therapeutic agents such as colchicine, etc. or the disease as a premalignant state. Therefore, Behcetʹs patients should be under special supervision in point of view of developing malignancies.