Tsunemine Hiroko, Maruoka Hayato, Akasaka Hiroshi, Udaka Kengo, Yamamoto Kazuyo, Ishikawa Sakane Emiko, Hiramoto Nobuhiro, Ito Kiminari, Kodaka Taiichi, Itoh Tomoo, Takahashi Takayuki
Department of Hematology, Shinko Hospital, Japan.
Intern Med. 2013;52(8):901-5. doi: 10.2169/internalmedicine.52.8519. Epub 2013 Apr 15.
We encountered a rare case of cytotoxic molecule-positive and Epstein-Barr virus (EBV)-associated peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), which was clinically preceded by polymyositis. A 50-year-old woman with a 4-year history of steroid-refractory polymyositis developed ulcerative skin swelling on her left arm. A diagnosis of cytotoxic molecule (TIA-1)-positive and EBV-associated PTCL-NOS was made on the basis of immunohistochemical and molecular examinations of the biopsied brachial muscle. Combination chemotherapies were ineffective, with a fatal outcome. Reassessment of the biopsy specimens of the muscle taken at the age of 46 years showed that the PTCL was already present, indicating that the polymyositis was likely a paraneoplastic manifestation.
我们遇到了一例罕见的细胞毒性分子阳性且与爱泼斯坦-巴尔病毒（EBV）相关的外周T细胞淋巴瘤，非特指型（PTCL-NOS），其临床前期表现为多发性肌炎。一名有4年激素难治性多发性肌炎病史的50岁女性，其左臂出现溃疡性皮肤肿胀。基于对活检肱二头肌的免疫组化和分子检查，诊断为细胞毒性分子（TIA-1）阳性且与EBV相关的PTCL-NOS。联合化疗无效，最终导致患者死亡。对46岁时所取肌肉活检标本的重新评估显示，当时PTCL就已存在，这表明多发性肌炎可能是一种副肿瘤表现。
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