Epstein-Barr virus 関連Bリンパ球増殖性疾患を合併した成人T細胞白血病  [in Japanese] Epstein-Barr virus-associated B cell lymphoproliferative disorder complicated in adult T-cell leukemia  [in Japanese]

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成人T細胞白血病(ATL)では高度の細胞性免疫不全を伴うが,EBウイルス関連リンパ増殖性疾患(EBV-LPD)を併発した報告は少ない。症例は53歳男性。両肺の多発結節陰影,体幹部の皮疹,鼠径部のリンパ節腫大,高カルシウム血症,ならびに末梢血に分葉核をもつ異常リンパ球が認められたため入院。抗HTLV-1抗体は陽性を示し,末梢血異常リンパ球はCD2, CD3, CD25陽性,CD4, CD8陰性であり,HTLV-I provirusの単クローン性組み込みが認められた。左鼠径部リンパ節生検では異常T細胞のほか,多クローン性のEBER陽性大型異常B細胞を認めた。以上より,EBV-LPDを合併した急性型ATLと診断した。化学療法によりATLの腫瘍量の減少が得られたが,入院第68病日より突然の高熱とともに,代謝性アシドーシス,多臓器不全に至り永眠された。死亡直前の末梢血では,EBウイルス量の増加が認められ,EBV-LPDの悪化が死亡原因と考えられた。

Adult T-cell leukemia (ATL) is accompanied by remarkably impaired cellular immune responses. Reports on the simultaneous development of ATL and Epstein-Barr virus-associated lymphoproliferative disorders (EBV-LPD) are, however, quite rare. A 53-years-old man was admitted to our hospital because of multiple bilateral pulmonary nodules, skin rash, lymphadenopathy, and hypercalcemia. The smear preparation of peripheral blood demonstrated pathological lymphocytes with lobulated nuclei. They expressed CD2, CD3, and CD25, but did not express either CD4 or CD8. In addition, he was positive for Human T-cell leukemic virus type-I (HTLV-I), and monoclonal integration of HTLV-I provirus was detected in peripheral blood. Unexpectedly, inguinal lymph node biopsy showed polyclonal proliferation of EBV-positive large and bizarre-shaped B cells, and monoclonal growth of large abnormal T cells. He was diagnosed as having acute type ATL complicated by EBV-LPD. Chemotherapy for ATL effectively decreased the tumor burden of ATL, but persistent high fever developed suddenly on the 68<sup>th</sup> day after admission, and he died of multi-organ failure with severe metabolic acidosis. At the time of multi-organ failure, viral load had increased strikingly, suggesting that the progression of EBV-LPD might have been responsible for multi-organ failure in this case.


  • Rinsho Ketsueki  

    Rinsho Ketsueki 49(4), 263-269, 2008-04-30 

    The Japanese Society of Hematology

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