A 3-year consecutive survey on current status of acquired inhibitors against coagulation factors in Japan

わが国における後天性凝固因子インヒビター患者の実態を把握する目的で3年間にわたるアンケート調査を行った．42施設から56例が登録されたが，うち55例が後天性血友病Aであり，その年齢分布は12-85（中央値70）歳であった．基礎疾患は自己免疫疾患と悪性腫瘍がそれぞれ17％を占め，出血症状は皮下出血や筋肉内出血の他，全経過中に重篤な出血が11％にみられた．インヒビターの最高値は1.1-758（中央値29.5）Bethesda 単位/mlであり，診断時に第VIII因子活性が同時に検出されたものが54％を占めた．止血療法はバイパス製剤の使用が多く，免疫抑制療法はプレドニゾロンの単独または他の免疫抑制剤との併用が多かった．経過を追跡しえた40例中21例でインヒビターが消失したが，9例は寛解に至らず，10例は死亡した．予後に影響を及ぼす因子として，止血療法，免疫抑制療法に対する反応性ならびに，経過中の感染症合併の有無で有意差がみられ，本症の治療に際しては，止血管理とともに感染症のコントロールが重要と思われた．

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