Der Hautarzt

, Volume 55, Issue 7, pp 658–663 | Cite as

„Smouldering systemic mastocytosis“

Erfolgreiche Therapie mit Cladribin
  • V. Schleyer
  • S. Meyer
  • M. Landthaler
  • R.-M. Szeimies
Kasuistik

Zusammenfassung

Mastozytosen sind eine heterogene Gruppe von Erkrankungen, die durch Proliferation und Akkumulation von Mastzellen in der Haut und in inneren Organen gekennzeichnet sind. Man unterscheidet die kutanen Mastozytosen von den systemischen Formen, die mit oder ohne Hautveränderungen auftreten können, vom Mastzellsarkom sowie den extrakutanen lokalisierten benignen Mastozytomen. Bei den systemischen Mastozytosen sind neben der Haut v. a. das Knochenmark, der Gastrointestinaltrakt, das Skelettsystem, Lymphknoten, Milz und Leber betroffen. Während die indolenten Formen der systemischen Mastozytose symptomatisch v. a. mit Antihistaminika, Glukokortikosteroiden und PUVA-Therapie behandelt werden, sind bei einem aggressiveren Verlauf bis hin zur Mastzellleukämie auch zytostatische Therapien erforderlich. Wir berichten über einen 53-jährigen Patienten mit beginnender „smouldering systemic mastocytosis“, der nach erfolglosen Therapieversuchen unter anderem mit hoch dosierten systemischen Glukokortikosteroiden und Interferon-α eine deutliche Besserung des Hautbefundes, einen signifikanten Rückgang der Serumtryptase und eine Konstanz der Knochenmarksinfiltration unter Gabe von Cladribin zeigte.

Schlagwörter

Mastozytose Urticaria pigmentosa Cladribin Glukokortikosteroide Interferon-α 

“Smoldering systemic mastocytosis”

Successful therapy with cladribine

Abstract

Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs. They are subdivided into cutaneous mastocytoses; systemic forms , which may appear with or without skin lesions; mast cell sarcomas and extracutaneous, localized, benign mastocytomas. Systemic mastocytoses apart from the skin mainly involve bone marrow, gastrointestinal tract, bones, lymph nodes, spleen and liver. Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy. A 53-year old patient with beginning “smoldering systemic mastocytosis” failed to respond to high-dose systemic glucocorticosteroids and interferon-α. Treatment with cladribine led to an impressive improvement of skin lesions, a significant decrease in tryptase serum levels and stabilization of bone marrow infiltrates.

Keywords

Mastocytosis Urticaria pigmentosa Cladribine Glucocorticosteroids Interferon-α 

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Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • V. Schleyer
    • 1
    • 2
  • S. Meyer
    • 1
  • M. Landthaler
    • 1
  • R.-M. Szeimies
    • 1
  1. 1.Klinik und Poliklinik für Dermatologie der Universität RegensburgRegensburg
  2. 2.Klinik und Poliklinik für Dermatologie der Universität RegensburgRegensburg

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