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ベーチェット病に続発した頭蓋内原発性悪性リンパ腫の1例について報告した。症例は43歳の女性で,不全型ベーチェット病によるブドウ膜炎の治療にcolchicineが使用された。その投与開始後6カ月目に小脳症状が出現し,生検と全身的検索から頭蓋内原発性悪性リンパ腫と診断された。免疫抑制剤の使用後に発生した悪性リンバ腫は,azathioprine, steroid, cyclophosphamide,さらにcyclosporinを使用した報告がほとんどである。本症例においては,広義の免疫抑制剤とされるcolchicineの投与がその発生に関与していた可能性も考えられた。
We reported a case of intracranial primary malignant lymphoma following Behçet disease treated with col-chicine.
A 43-year-old female with a past history of oral ulcer and folliculitis visited the Department of Ophthalmol-ogy on December, 1990, because of her impaired visual acuity. A diagnosis of uveitis due to incomplete type Behcet disease was made from funduscopic examination and her past history. She had been received 1.0mg/day colchicine for six months in the outpatient clinic. She was referred to our department on August 19, 1991,with nausea and headache. On admission, neurological exam-ination revealed slight right cerebellar ataxia showing dysmetria and dysdiadochokinesis. Computed tomogra-phy scan and magnetic resonance images demonstrated a malignant lymphoma in the left cerebellar hemi-sphere. Stereotaxic biopsy resulted in the B cell type malignant lymphoma (Diffuse large cell type). Gallium-scintigraphy, physical examination and peripheral blood examination disclosed no systemic abnormalities. There was a reduction of lymphocyte's response to PHA (phytohemagglutinin) stimulation (1.24). The tubercu-lin reaction was negative. Colchicine was discontinued and the therapy with radiation and prednisolone (20mg/ day) was started under the diagnosis of intracranial primary malignant lymphoma. She was discharged without any neurological deficits on November 25, 1991. She still suffers from impaired visual acuity, but has no problems in daily life.
To our knowledge no intracranial primary malignant lymphoma following Behcet disease has been reported previously. The relationship between Behcet disease treated with colchicine and the occurrence of malignant lymphoma is discussed. In our case we speculated that malignant lymphoma might have occurred in an im-munosuppressive state due to administration colchicine.
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