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一名55岁患有胸腺瘤和低丙种球蛋白血症(古德综合征)、溃疡性结肠炎及巨细胞病毒感染的女性

[A 55-year-old woman with thymoma and hypogammaglobulinemia (Good syndrome), ulcerative colitis, and cytomegalovirus infection].

作者信息

Kahraman Alisan, Miller Michael, Maldonado-Lopez Evelyn, Baba Hideo A, Treichel Ulrich, Gerken Guido

机构信息

Klinik für Gastroenterologie/Hepatologie, Universitätsklinikum Essen, Essen, Germany.

出版信息

Med Klin (Munich). 2009 Feb 15;104(2):150-4. doi: 10.1007/s00063-009-1026-6. Epub 2009 Feb 26.

Abstract

CASE REPORT

The authors report on a 55-year-old female patient after R1 resection of a malignant thymoma with spindle type epithelial cells (WHO type A, Masaoka stage III) referred for further therapy of an ulcerative colitis. At that time, both adjuvant radiation and cytostatic therapy were not applicable due to severe activity of the ulcerative colitis. Under immunosuppressive treatment with azathioprine and steroids, the patient developed cytomegalovirus (CMV) enteritis which was triggered by therapy-induced leukopenia. After a switch from azathioprine to mycophenolatmofetil (MMF) treatment and administration of cidofovir because of nonresponse to ganciclovir and incompatibility of foscarnet sodium (Foscavir), the patient clinically improved. In addition, the patient was treated with immunoglobulins every 3-4 weeks because of antibody deficiency. At present, 3.5 years after R1 resection, the patient still has no clues of a remaining tumor mass under current immunosuppressive therapy. Ulcerative colitis is also in complete remission stage.

CONCLUSION

This case indicates the very rare features of a syndrome with thymoma and antibody deficiency which was first described by Robert Good. Furthermore, the impact of immunosuppressive therapy and management of opportunistic infections on the course of this disease is obvious.

摘要

病例报告

作者报告了一名55岁女性患者,该患者接受了R1切除手术,切除的是一例具有梭形上皮细胞的恶性胸腺瘤(世界卫生组织A型,马萨oka III期),之后因溃疡性结肠炎前来接受进一步治疗。当时,由于溃疡性结肠炎的严重活动,辅助放疗和细胞抑制疗法均不适用。在使用硫唑嘌呤和类固醇进行免疫抑制治疗期间,患者因治疗引起的白细胞减少症引发了巨细胞病毒(CMV)肠炎。在从硫唑嘌呤改用霉酚酸酯(MMF)治疗并因对更昔洛韦无反应且膦甲酸钠(福斯可韦)不相容而给予西多福韦后,患者临床症状有所改善。此外,由于抗体缺乏,患者每3 - 4周接受一次免疫球蛋白治疗。目前 在R1切除术后3.5年,在当前免疫抑制治疗下,患者仍未发现残留肿瘤肿块的迹象。溃疡性结肠炎也处于完全缓解期。

结论

本病例显示了一种由罗伯特·古德首次描述的胸腺瘤与抗体缺乏综合征的罕见特征。此外,免疫抑制治疗和机会性感染的管理对该疾病病程的影响是显而易见的。

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