血栓性血小板減少性紫斑病と血球貪食症候群を合併した全身性エリテマトーデスの1例 A case of systemic lupus erythematosus associated with thrombotic thrombocytopenic purpura and hemophagocytic syndrome

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  症例は,44歳女性.2002年8月多関節痛等出現.11月に多関節痛,白血球減少,抗ds-DNA抗体陽性,抗核抗体陽性,梅毒血清反応偽陽性などから全身性エリテマトーデス(SLE)の診断をうけた.ステロイドにて治療開始後も低補体血症続きコントロール不十分であった.2003年11月発熱,多関節痛,12月に肉眼的血尿,紫斑が出現.12月25日入院となった.破砕赤血球の存在,溶血,血小板減少性紫斑,頭痛,腎障害,発熱から血栓性血小板減少性紫斑病(TTP)を考えた.また,高フェリチン血症から血球貪食症候群(HPS)を疑い骨髄穿刺液検査を施行.マクロファージによる血球貪食像を認め血球貪食症候群も合併と診断した.メチルプレドニゾロンパルス療法を3日間,後療法にプレドニゾロン60 mgを開始.血漿交換も第1病日より開始した.血漿交換終了後TTPの再燃あり.血漿交換追加にて症状改善した.本症例ではvWF-CP (ADAMTS-13)活性の低下がみられ,抗vWF-CP抗体は陽性であった.TTP, HPSは共にSLEの重要な難治性合併症である.両病態が同時に合併しうることを念頭に置き,迅速に診断することが救命につながる.<br>

  The case: A 44-year-old female. Developed polyarthralgia in August 2002. The patient was diagnosed with systemic lupus erythematosus (SLE) in November, due to polyarthralgia, leukopenia, anti ds-DNA antibody positive, antinuclear antibody positive, and false positive serologic test for syphilis. Hypocomplementemia continued even after the steroid treatment was conducted with insufficient control. In November 2003, ran fever and observed polyarthralgia. In December, gross hematuria and purpura appeared. The patient was hospitalized on December 25. Thrombotic thrombocytopenic purpura (TTP) was suspected from the emergence of fragmented red cells, hemolysis, thrombocytopenic purpura, headache, renal dysfunction and fever. As hemophagocytic syndrome (HPS) was suspected from hyper-ferritinemia, bone marrow aspiration was conducted. Macrophage confirmed hemophagocytic image and the patient was diagnosed with HPS complication. Methylprednisolone pulse therapy was conducted for three days, followed by the administration of prednisolone 60 mg. Plasma exchange therapy was also conducted from the first day of hospitalization. Recurrence of TTP after plasma exchange therapy, but it improved by additional plasma exchange. Low vWF-CP (ADAMTS-13) activity was observed in this case, and anti-vWF-CP antibody was positive. TTP and HPS are both critical intractable complications of SLE. Bearing in mind the possibility of simultaneous complication of both symptoms, prompt diagnosis is crucial for life-saving.<br>

収録刊行物

  • 日本臨床免疫学会会誌 = Japanese journal of clinical immunology  

    日本臨床免疫学会会誌 = Japanese journal of clinical immunology 29(6), 384-388, 2006-12-31 

    The Japan Society for Clinical Immunology

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各種コード

  • NII論文ID(NAID)
    10018714115
  • NII書誌ID(NCID)
    AN00357971
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    09114300
  • データ提供元
    CJP書誌  J-STAGE 
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