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Hepatitis-assoziierte Kryoglobulinämie

Hepatitis associated cryoglobulinemia

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Zusammenfassung

Kryoglobuline sind Immunglobuline, die bei Temperaturen unter 37°C präzipitieren. Klinisch manifestiert sich eine Kryoglobulinämie mit sehr unterschiedlichen Symptomen an diversen Organen. Die klinisch bedeutendsten Symptome sind Fatigue, periphere Polyneuropathie und vaskulitisassoziierte Hautdefekte. Pathophysiologisch beruht eine Kryoglobulinämie auf einer gestörten Immunkaskade mit einer gesteigerten B-Zell-Aktivität. Der oftmals beobachtete Übergang einer Kryoglobulinämie in ein malignes Non-Hodgkin-Lymphom ist fließend. Die Assoziation zwischen Kryoglobulinämie und Virushepatitis ist am größten bei der Hepatitis-C-Virusinfektion. Andere Ursachen einer Kryoglobulinämie sind rheumatische und hämatologische Erkrankungen. Während früher v. a. mit Plasmapherese und Immunsuppression therapiert wurde, hat sich, durch die hohe Korrelation mit einer HCV-Infektion, die Bedeutung antiviraler Therapien im Rahmen von Kryoglobulinämien herausgestellt. Fälle von rascher Verschlechterung unter Interferon α wurden jedoch auch beobachtet. Eine vielversprechende alternative Therapieoption ist der Einsatz von Rituximab.

Abstract

Cryoglobulins are immunoglobulins that precipitate at temperatures below 37°C. Clinically cryoglobulinemia is manifested in a variety of symptoms on different organs. The most important clinical symptoms are fatigue, peripheral neuropathy and vasculitis associated skin lesions. Pathophysiologically cryoglobulinemia is based on a disturbed immunocascade with an elevated B-cell-activity. Often a cryoglobulinemia progresses smoothly to a Non-Hodgkin-Lymphoma. The main activator of a cryoglobulinemia is a Hepatitis C virus infection. Other causes for developing a cryoglobulinemia are rheumatological and haematological diseases. In the past cryoglubulinemia has predominantly been treated with plasmapheresis and immunosuppression, nowadays antiviral strategies are becoming more important. Cases of rapid worsening under therapy with interferon alpha have also been reported. A promising new option is the use of rituximab.

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  1. Abteilung Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Carl-Neuberg-Straße 1, 30625, Hannover, Deutschland

    S. Pischke, M. Cornberg &  M.P. Manns

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  1. S. Pischke
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  2. M. Cornberg
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  3. M.P. Manns
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Pischke, S., Cornberg, M. & Manns, M. Hepatitis-assoziierte Kryoglobulinämie. Internist 49, 297–304 (2008). https://doi.org/10.1007/s00108-007-2014-7

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