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Husten, Atemnot und B-Symptome bei einer 40-jährigen Frau


Wolfensberger, A; Benz, R; Russi, E W; Tinguely, M; Keller, C; Gerber, B (2013). Husten, Atemnot und B-Symptome bei einer 40-jährigen Frau. Der Internist, 54(8):1011-1015.

Abstract

Multicentric Castleman's disease (MCD) is a rare polyclonal lymphoproliferative disorder that is typically accompanied by an overproduction of circulating cytokines (mainly interleukin-6). We here report the case of a 40-year-old HIV-negative woman with pulmonary manifestation of MCD. There is no standard treatment for MCD. In our patient, various treatment courses with immunomodulatory drugs were unsuccessful. Finally, treatment with the interleukin-6 receptor antibody tocilizumab has resulted in continual clinical improvement over the last 5 years.

Der Morbus Castleman ist eine seltene polyklonale, lymphoproliferative Erkrankung, bei der Mediatoren von Entzündungsreaktionen, v. a. Interleukin-6, eine wichtige pathophysiologische Rolle spielen. Zur Behandlung dieser Krankheit ist keine Standardtherapie etabliert. Wir berichten über den Fall einer 40-jährigen HIV-negativen Patientin mit primär pulmonaler Manifestation eines HHV-8-negativen, plasmazellreichen multizentrischen Morbus Castleman. Verschiedene Therapieversuche mit Immunmodulatoren wurden durchgeführt, bevor eine Behandlung mit dem Interleukin-6-Rezeptor-Antikörper Tocilizumab begonnen wurde. Seit 5 Jahren ist der klinische Verlauf unter fortgesetzter Tocilizumabgabe stabil.

Abstract

Multicentric Castleman's disease (MCD) is a rare polyclonal lymphoproliferative disorder that is typically accompanied by an overproduction of circulating cytokines (mainly interleukin-6). We here report the case of a 40-year-old HIV-negative woman with pulmonary manifestation of MCD. There is no standard treatment for MCD. In our patient, various treatment courses with immunomodulatory drugs were unsuccessful. Finally, treatment with the interleukin-6 receptor antibody tocilizumab has resulted in continual clinical improvement over the last 5 years.

Der Morbus Castleman ist eine seltene polyklonale, lymphoproliferative Erkrankung, bei der Mediatoren von Entzündungsreaktionen, v. a. Interleukin-6, eine wichtige pathophysiologische Rolle spielen. Zur Behandlung dieser Krankheit ist keine Standardtherapie etabliert. Wir berichten über den Fall einer 40-jährigen HIV-negativen Patientin mit primär pulmonaler Manifestation eines HHV-8-negativen, plasmazellreichen multizentrischen Morbus Castleman. Verschiedene Therapieversuche mit Immunmodulatoren wurden durchgeführt, bevor eine Behandlung mit dem Interleukin-6-Rezeptor-Antikörper Tocilizumab begonnen wurde. Seit 5 Jahren ist der klinische Verlauf unter fortgesetzter Tocilizumabgabe stabil.

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Additional indexing

Other titles:Dyspnoea, cough and B symptoms in a 40-year-old woman
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Hematology
04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Infectious Diseases
Dewey Decimal Classification:610 Medicine & health
Language:German
Date:2013
Deposited On:23 Sep 2013 08:24
Last Modified:05 Apr 2016 16:59
Publisher:Springer
ISSN:0020-9554
Publisher DOI:https://doi.org/10.1007/s00108-013-3327-3
PubMed ID:23836253

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