両眼の閉塞性網膜血管炎を契機に診断され急速な転帰をたどった悪性リンパ腫の1例

Malignant lymphoma with bilateral occlusive retinal vasculitis

要約　背景：閉塞性網膜血管炎は，ぶどう膜炎や脈絡膜炎などの眼炎症疾患以外にも全身の炎症性疾患や感染などの関与を想定し全身精査を行うことが重要であると示唆されているが，悪性疾患による閉塞性網膜血管炎の報告は稀である。今回，両眼の閉塞性網膜血管炎を契機に悪性リンパ腫と診断され，急な転帰をたどった症例を経験したので報告する。

症例：患者は65歳，男性。数日間継続する両眼の羞明を主訴に前医を受診し，両眼の網膜動脈分枝閉塞症と診断され川崎市立川崎病院（以下，当院）を紹介され受診した。初診時視力は右（0.8），左（1.2）で，光干渉断層計では両眼に網膜内層の浮腫を認め，蛍光眼底造影検査では両眼の末梢血管に多発する閉塞性網膜血管炎を認めた。同時期に睾丸痛，排尿困難，両足底の痺れの自覚があり，悪性疾患の可能性を否定するため当院内科に入院のうえで全身精査を行った。その後，CTで全身に複数のリンパ節の腫大を認め，頸部リンパ節生検によりびまん性大細胞型B細胞リンパ腫と診断され，化学療法（R-CHOP療法/メトトレキサート大量髄注）が開始された。治療中に四肢麻痺と徐呼吸が生じ，頭部MRIで小脳，頭頂葉，延髄に多発する小梗塞を認めた。入院9日目のベッドサイドでの眼底検査では網膜浮腫の改善を認めたが，その後14日目には右眼底耳側に軟性白斑の出現を認めた。入院15日目に意識レベルが低下し，16日目に死亡した。

結論：両眼で多発する閉塞性網膜血管炎は稀であり，本症例では眼炎症疾患以外にも全身疾患の関与を想定し早期に全身精査を行ったことで悪性リンパ腫と確定診断することが可能であった。病勢が強く最終的には救命には至らなかったが，両眼の閉塞性網膜血管炎がみられた場合，全身の悪性疾患の可能性も考慮し早期の全身精査を施行することが重要であると示唆された。

Abstract　Background：In addition to ocular inflammatory diseases such as uveitis and choroiditis, systemic diseases, such as inflammatory diseases and infections may be involved in the development of retinal vasculitis；thus, performing a systemic examination is important. Retinal vasculitis caused by malignant diseases has been reported, but its incidence is rare. We experienced a case of malignant lymphoma diagnosed as a result of occlusive retinal vasculitis in both eyes, and the patient underwent a rapid transformation.

Case：The patient was a 65-year-old man who visited the previous doctor with complaints of photophobia in both eyes for several days. He was referred to our hospital with a diagnosis of branch retinal artery occlusion in both eyes. At the time of the visit, his best corrected visual acuity was 0.8 in the right eye and 1.2 in the left eye. Optical coherence tomography（OCT）showed edema in the inner retinal layer, and fluorescence fundus angiography showed multiple occlusive retinal vasculitis in the periphery of both eyes. At the same time, the patient experienced testicular pain, urinary retention, and plantar numbness, and was admitted for systemic examination to rule out the possibility of malignant disease. A computed tomography（CT）scan revealed enlarged lymph nodes, and a biopsy revealed diffuse large B-cell lymphoma, for which chemotherapy（R-CHOP therapy/methotrexate high-dose intrathecal injection）was initiated. During the treatment, the patient developed tetraplegia and slow breathing, and a head MRI showed multiple small infarcts in the cerebellum, parietal lobe, and medulla oblongata. A bedside fundus examination on the 9th day of hospitalization showed improvement of retinal edema, but on the 14th day of hospitalization, soft white spots appeared on the right fundus. The patient's level of consciousness decreased on the 15th day of hospitalization and he died on the 16th day.

Conclusion：In this case, early systemic examination was performed suspecting the involvement of systemic diseases in addition to ocular inflammatory diseases, which led to the diagnosis of malignant lymphoma. Although the disease was life-threatening and the patient died from malignant lymphoma, it suggests the importance of early systemic examination considering the possibility of systemic malignant diseases when occlusive retinal vasculitis is observed in both eyes.