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57歳男性が,半年前からの両眼眼瞼下垂を主訴として受診した。その2か月前から発熱と呼吸困難があり,さらに8年前から耳下腺と顎下腺の腫脹があった。両眼瞼の涙腺部と唾液腺部が腫脹し,結膜円蓋部に乳頭様の小胞があり,全身のリンパ節が腫大していた。結膜生検と気管枝鏡による肺生検の病理像は,diffuse medium-sized cell type, intermediate lymphocytic lymphoma subtypeであった。免疫学的表現型は,CD5(+) CD10(−)であり,染色体はt (11;14)であった。これらからmantle zone lymphomaと診断された。副腎皮質ステロイド製剤と複数の抗癌剤の全身投与で,全身状態と眼症状は寛解した。
A 57-year-old male presented with bilateral blepharoptosis of 6 months' duration. Fever and dyspnea had been present since 8 months before. He had noted swelling of parotid and submandibular glands for 8 years. He manifested, bilaterally, swollen lacrimal and salivary glands. Small papillary vesicules were present in the upper conjunctival fornices. Biopsy of the conjunctiva and the lung showed intermediate lymphocytic lymphoma (diffuse medium-sized cell type). The surface marker of tumor cells showed CD5 (+) CD10 (-). Chromosomal analysis of lymphoma cells showed t (11;14) (q13;q32). These findings led to the diagnosis of mantle zone lymphoma. Systemic treatments with corticosteroids and cytostatic agents led to improvement in ocular and systemic findings.
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