視神経浸潤の後に眼窩浸潤をきたしたT細胞性急性リンパ性白血病の1剖検例

An autopsied case with orbital infiltration of leukemic cells after optic nerve invasion by acute T-cell leukemia

要約　目的：T細胞性急性リンパ性白血病（T-ALL）が視神経と眼窩に浸潤した症例の報告。

症例：患者は46歳の男性．2014年にT-ALLと診断されたが，化学療法により完全寛解を得た。診断7か月後に右眼霧視出現。T-ALLの再発と診断。右眼は網膜中心動脈閉塞症のため失明した。再寛解後に末梢血幹細胞移植を施行したが，3か月後に再発。左眼視力障害，眼球突出が出現。治療により症状は一時軽快したが，左眼失明。診断19か月後に死亡し，病理解剖を行った。

所見：全身にT-ALL浸潤があり，網脈絡膜，視神経乳頭，眼窩脂肪組織，外眼筋にT-ALL細胞が認められた。

結論：T-ALLの眼病理組織の報告は希少であり，報告した。

Abstract　Purpose：To report a case of T-cell acute lymphoblastic leukemia（T-ALL）with optic nerve and orbital invasion.

Case：A 46-year-old man was diagnosed as T-ALL in 2014, received chemotherapy, and obtained complete remission（CR）. T-ALL relapsed with blurring vision of right eye 7months after diagnosis. He lost vision in the right eye due to central retinal artery occlusion. 2nd CR was achieved, then peripheral blood stem cell transfusion（PBSCT）was operated. However 2nd relapse was detected in meninges only 3 months after the PBSCT, presenting paropsia of left eye was progressed to proptosis and ophthalmalgia. Orbital MRI scan showed contrast enhancement in bilateral extraocular muscle, left orbital fat, and left optic nerve. After treatment, proptosis was regressed transiently. Despite he died by disease progress 19 months after diagnosis.

Findings：Autopsy revealed systemic T-ALL involvement including eyeball and orbit. The immunostaining pattern was positive for CD3, MIC2, negative for CD20. Coagulation necrosis of optic nerve and involvement of T-ALL in orbital fat, uvea, retina and extraocular muscle were observed.

Conclusion：An autopsy report indicating involvement of orbital mass with T-ALL is extremely rare, and regarded as worthy information.