漿液性網膜剝離で初発し血栓性血小板減少性紫斑病と判明した1例

A case of thrombotic thrombocytopenic purpura with serous retinal detachment as the initial manifestation

要約　51歳男性が両眼に変視症を自覚し，その当日に受診した。矯正視力は右0.8，左0.9であり，右眼に＋0.75D，左眼に＋2.0Dの遠視があった。両眼に乳頭から黄斑部に及ぶ境界鮮明な漿液性網膜剝離があった。蛍光眼底造影で造影早期に点状の過蛍光，後期に色素貯留が網膜剝離下にあり，原田病を疑った。血液検査で，血小板が6,000/μlと低く，溶血性貧血，肝機能障害，腎機能障害があり，塗抹標本で破砕赤血球があった。その後昏睡状態になり，血栓性血小板減少性紫斑病（thrombotic thrombocytopenic purpura：TTP）と診断した。6回の血漿交換療法を行い，意識と眼底所見が回復し，両眼とも1.2の視力を得た。本症例は，漿液性網膜剝離で初発し，その後TTPと判明した初めての報告である。

Abstract.　A 51-year-old man presented with metamorphopsia in his both eye since the same morning. His corrected visual acuity was 0.8 right and 0.9 left. Both eyes were hyperopic with＋0.75D right and＋2.0D left. Both eyes showed serous retinal detachment extending from the disc to the macular area. Fluorescein angiography showed hyperfluorescent dots in the early phase and dye pooling posterior to the detached retina in the late phase. The findings were suggestive of Harada disease. Hematological studies showed decrease of thrombocytes to 6,000/μl. He had also hemolytic anemia and hepatorenal dysfunction. These findings led to the diagnosis of thrombotic thrombocytopenic purpura（TTP）. His ocular and systemic symptoms recovered completely following 6 sessions of plasmapheresis. To our best knowledge,this is the first reported case of serous retinal detachment as the initial manifestation of TTP.