末梢神経障害を呈しVirus-Associated Hemophagocytic Syndrome様の所見を伴った大型穎粒リンパ球性白血病

Peripheral Neuropathy in Large Granular Lymphocytic Leukemia

　症例は，両下肢のしびれ感を主訴として入院した16歳女性である。慢性活動性EBウイルス感染症を基礎に，発熱，肝脾腫，汎血球減少および肝機能障害など，Virus-associated hemophagocytic syndrome（VAHS）を呈し，大型顆粒リンパ球（Large granular lymphocyte：LGL）性白血病が認められた。末梢神経障害は進行性で，両膝以下の表在感覚の消失，筋萎縮，筋力低下，深部腱反射の消失をきたし，遅れて上肢にも同様の変化が出現した。髄液中では，蛋白，細胞数の増加が認められ，マクロファージの活性化の指標となるネオプテリンの著増も認められ，本症例での末梢神経障害の機序として活性化されたマクロファージによる可能性が考えられた。

A 16-year old woman with LGL leukemia deve-loped peripheral neuropathy. She showed virus-as-sociated hemophagocytic syndrome (VAHS)-like signs including high fever, liver dysfunction, huge splenomegaly, hepatomegaly and pancytopenia. The presence of chronic active EB virus infection was proved by marked high titers for IgG and IgA antibodies to the Epstein-Barr viral capsid and early antigens and low titers of antibody to Epstein -Barr nuclear antigens. She showed dysesthesia and paresthesia of bilateral lower extremities with marked swelling and tenderness, and later developed muscular weakness and atrophy with areflexia of lower extremities. Findings of the cen-tral nervous system dysfunction were not observed except for the acceleraion of jaw jerk. Pleocytosis and increased protein levels in the cerebrospinal fluid were found. Pulse therapy of methyl - predo-nisolone and high dose intravenous immunoglobulin therapy (20 g/day for 3 days) were effective for neu-rological findings. The incereased neopterin in the cerebrospinal fluid suggested that peripheral neuro-pathy was caused by activated macrophages.