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55-jährige Patientin mit Thymom und Hypogammaglobulinämie (Good-Syndrom), Colitis ulcerosa sowie CMV-Infektion

A 55-Year-Old Woman with Thymoma and Hypogammaglobulinemia (Good Syndrome), Ulcerative Colitis, and Cytomegalovirus Infection

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Zusammenfassung

Fallbeschreibung:

Die Autoren berichten über eine 55-jährige Patientin nach erfolgter R1-Resektion eines Thymoms vom Spindelzelltyp (Typ A nach WHO, Masaoka-Stadium III), die ihnen zur weiteren Therapie einer Colitis ulcerosa zugewiesen wurde. Eine adjuvante Radiochemotherapie war aufgrund der ausgeprägten Aktivität der Colitis ulcerosa vorerst nicht vertretbar. Unter immunsuppressiver Behandlung mit Azathioprin und Steroiden entwickelte die Patientin eine Zytomegalievirus-(CMV-)Enteritis, die durch die azathioprininduzierte Leukopenie begünstigt worden war. Unter Umstellung der Immunsuppression von Azathioprin auf Mycophenolatmofetil (MMF) sowie der Gabe von Cidofovir aufgrund einer Ganciclovirrefraktärität der CMV-Ulzera und einer gleichzeitigen Foscarnet-Natrium-(Foscavir®-)Unverträglichkeit der Patientin kam es zur raschen Besserung der Klinik. Zusätzlich erhielt die Patientin aufgrund ihres Antikörpermangelsyndroms Immunglobuline in 3- bis 4-wöchigen Abständen. Mittlerweile 3,5 Jahre nach erfolgter R1-Resektion ist die Patientin unter Immunsuppression mit MMF weiterhin ohne Anhalt für ein Rezidiv des Thymoms. Zudem zeigt sich auch die Colitis ulcerosa in Remission.

Schlussfolgerung:

Dieser Fallbericht veranschaulicht eine seltene Erkrankung aus Thymom und Antikörpermangelsyndrom, welche erstmalig von Robert Good beschrieben wurde, und weist auf die Wichtigkeit der immunsuppressiven Behandlung und der Beherrschung opportunistischer Infektionen hin.

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.

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Correspondence to Guido Gerken.

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Kahraman, A., Miller, M., Maldonado-Lopez, E. et al. 55-jährige Patientin mit Thymom und Hypogammaglobulinämie (Good-Syndrom), Colitis ulcerosa sowie CMV-Infektion. Med Klin 104, 150–154 (2009). https://doi.org/10.1007/s00063-009-1026-6

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  • DOI: https://doi.org/10.1007/s00063-009-1026-6

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