慢性再発性の経過を辿りetoposideが有効であった血球貧食症候群の1例 Successful use of etoposide in an elderly patient with chronic recurrent hemophagocytic syndrome

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症例は66歳, 男性. 1993年に食道癌に対し食道再建術施行. 1997年6月一過性の発熱が出現, 8月再び39℃台の発熱とLDHの上昇を認めた. その後も原因不明の発熱を繰り返し血球減少も出現したため, 1998年1月19日当科入院となった. 体温38.5℃, 表在リンパ節や肝脾触知せず. 皮疹はみられなかった. Hb 8.6g/dl, 血小板7.9万/μlと血球減少を認め, LDH 1,606IU/l, フェリチン>3,000ng/mlと異常高値を示した. 骨髄は低形成を示し, 成熟血球を貪食した組織球の著明な増加を認めた. 芽球の増加や異常細胞の出現はみられなかった. 抗核抗体は陰性でEBウイルス等のウイルス抗体価の有意な上昇は認めなかった. 以上より血球貪食症候群 (hemophagocytic syndrome, 以下HPS) と診断したが原疾患は不明であった. また, 胸水を伴う間質性肺炎も併発し低酸素血症を示した. γ-グロブリン製剤やメチルプレドニゾロン大量療法を行うも効果なく, etoposide (VP-16, 150mg/日) を投与したところHPSの寛解が得られ, 同時に間質性肺炎も改善した. その後, 2001年7月再び発熱, 血球減少が出現し, 骨髄穿刺検査にてHPSの再発と判断しVP-16の再投与を行ったところ改善が得られた. 本例は基礎疾患不明のHPSで, 慢性再発性の経過を辿り, 間質性肺炎を併発し, VP-16が著効するなど興味ある症例と考えられた.

A 66-year-old man was admitted to our hospital for fever on January 19, 1998. He began showing periodic high fever in June 1997 and an increased serum LDH in August 1997. His history included surgery for esophageal cancer in 1993. On admission, the patient's body temperature was 38.5°C. Physical examination was negative for lymphadenopathy, hepatosplenomegaly, and skin rash. Peripheral blood revealed a hemoglobin level of 8.6g/dl and a platelet count of 7.9×104/μl. Bone marrow examination showed hypocellularity with marked histiocytic hemophagocytosis. The various bacterial cultures were negative. Serum LDH was elevated to 1, 606IU/l, and ferritin was greater than 3, 000ng/ml. Antinuclear antibodies were negative. No significant elevation of viral antibody titers including that to Epstein-Barr virus was found. Hemophagocytic syndrome (HPS) was diagnosed, but no underlying diseases was identified. The patient's condition was complicated by interstitial pneumonia and pleural effusion. γ-globulin and pulse methylprednisolone both proved ineffective for the HPS; however, complete remission was achieved with cyclic intravenous administration of etoposide (VP-16, 150mg/day). Interestingly, the interstitial pneumonia resolved promptly with etoposide therapy. The patient relapsed, in July 2001, exhibitting high fever, cytopenia, and marrow hemophagocytosis. His condition was ameliorated by administration of etoposide.This was a rare case of chronic and recurrent HPS of unknown etiology accompanied by interstitial pneumonia. Etoposide should be considered as a primary therapy for HPS and its comlications in cases such as our patients.

収録刊行物

  • 日本老年医学会雑誌

    日本老年医学会雑誌 40(2), 160-166, 2003-03-25

    一般社団法人 日本老年医学会

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各種コード

  • NII論文ID(NAID)
    10010768266
  • NII書誌ID(NCID)
    AN00199010
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    03009173
  • NDL 記事登録ID
    6536298
  • NDL 雑誌分類
    ZS21(科学技術--医学--内科学)
  • NDL 請求記号
    Z19-25
  • データ提供元
    CJP書誌  NDL  J-STAGE 
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