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噬血细胞综合征后发生的骨骼肌T细胞淋巴瘤

[Skeletal muscle T-cell lymphoma following hemophagocytic syndrome].

作者信息

Yamaguchi H, Hashimoto M, Mizuki T, Nakamura H, Nakayama K, Nakamura K, Tajika K, Inokuchi K, Dan K

机构信息

Department of Internal Medicine, Nippon Medical School.

出版信息

Rinsho Ketsueki. 2000 Sep;41(9):712-7.

Abstract

A 43-year-old man was admitted to our hospital because of hemophagocytic syndrome (HPS) in August, 1998. A CT scan, gallium scintigraphy, gastrofiberscopy and colonofiberscopy showed no evidence of malignant lymphoma. Virus-associated HPS was suspected because of an increased titer of anti-Epstein-Barr (EB) virus antibody (EBV VCA IgG 2,560x, EBV EA IgG 40x, EBNA 20x). The HPS resolved spontaneously for 40 hospital days, but two weeks into the period of HPS remission, the patient developed pain and marked swelling of the right thigh muscle, and pectoral, biceps brachii, quadriceps femoralis and masseter muscles. Otherwise, CT scan and gallium scintigraphy showed no abnormal findings. A biopsy of the right quadriceps femoralis muscle revealed non-Hodgkin's lymphoma with muscle infiltration. Immunohistologic examination confirmed T-cell type (CD3, CD43, CD45, CD45RO) lymphoma, and Southern blot analysis for T-cell receptor revealed a rearranged band. The lymphoma cells were negative for EBV genome monoclonality. The patient responded well to CHOP therapy and achieved a complete remission. This is considered a very rare case of T-cell lymphoma infiltrating multiple skeletal muscles following an episode of hemophagocytic syndrome.

摘要

1998年8月,一名43岁男性因噬血细胞综合征(HPS)入住我院。CT扫描、镓扫描、胃镜检查和结肠镜检查均未发现恶性淋巴瘤的证据。由于抗Epstein-Barr(EB)病毒抗体滴度升高(EBV VCA IgG 2560倍、EBV EA IgG 40倍、EBNA 20倍),怀疑为病毒相关性HPS。HPS在住院40天时自发缓解,但在HPS缓解期两周后,患者出现右大腿肌肉以及胸肌、肱二头肌、股四头肌和咬肌疼痛和明显肿胀。除此之外,CT扫描和镓扫描未发现异常。右股四头肌活检显示为非霍奇金淋巴瘤伴肌肉浸润。免疫组织学检查证实为T细胞型(CD3、CD43、CD45、CD45RO)淋巴瘤,T细胞受体的Southern印迹分析显示有重排条带。淋巴瘤细胞的EBV基因组单克隆性为阴性。患者对CHOP治疗反应良好,实现了完全缓解。这被认为是一例非常罕见的噬血细胞综合征发作后T细胞淋巴瘤浸润多个骨骼肌的病例。

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