Takahashi Tohru, Maruyama Yumiko, Saitoh Mayuko, Itoh Hideto, Yoshimoto Mitsuru, Tsujisaki Masayuki
Department of Hematology, Tenshi Hospital, Kita-12, Higashi-3-1-1, Higashi-ku, Sapporo 065-0012, Japan.
Department of Gastroenterology, Tenshi Hospital, Kita-12, Higashi-3-1-1, Higashi-ku, Sapporo 065-0012, Japan.
Case Rep Hematol. 2015;2015:926874. doi: 10.1155/2015/926874. Epub 2015 Sep 3.
Epstein-Barr virus (EBV) associated lymphoproliferative disorder (LPD) after immunosuppressive therapy for aplastic anemia (AA) is extremely rare in a nontransplant setting and has not been well described. This report describes a severe AA patient in whom fatal EBV-LPD developed after being treated with rabbit antithymocyte globulins (ATG) and cyclosporine A (CsA). An 81-year-old man was diagnosed as having severe AA. He was started on CsA followed by administration of ATG for five consecutive days. One month after the start of ATG, persistent fever which was not responsive to antibiotics or antifungal agents developed and atypical lymphocytes emerged in peripheral blood. Repeated blood cultures were negative. An extremely high level of EBV virus in his peripheral blood plasma was detected by means of a quantitative real-time PCR assay. Even after the cessation of CsA, the fever persisted and the peripheral atypical lymphocytes proliferated rapidly. The patient suffered from respiratory failure, liver dysfunction, and metabolic acidosis. Rituximab was administered without success and he died.
再生障碍性贫血（AA）免疫抑制治疗后发生的与 Epstein-Barr 病毒（EBV）相关的淋巴增殖性疾病（LPD）在非移植环境中极为罕见，且尚未得到充分描述。本报告描述了一名严重 AA 患者，其在接受兔抗胸腺细胞球蛋白（ATG）和环孢素 A（CsA）治疗后发生了致命的 EBV-LPD。一名 81 岁男性被诊断为严重 AA。他开始接受 CsA 治疗，随后连续五天给予 ATG。ATG 开始使用一个月后，出现持续发热，对抗生素或抗真菌药物均无反应，外周血中出现非典型淋巴细胞。多次血培养均为阴性。通过定量实时 PCR 检测发现其外周血浆中 EBV 病毒水平极高。即使停用 CsA 后，发热仍持续，外周非典型淋巴细胞迅速增殖。患者出现呼吸衰竭、肝功能障碍和代谢性酸中毒。给予利妥昔单抗治疗无效，患者死亡。
