Dtsch Med Wochenschr 2014; 139(30): 1523-1538
DOI: 10.1055/s-0034-1370055
CME | Review article
© Georg Thieme Verlag KG Stuttgart · New York

Systemische Mastzellaktivierungserkrankung: Ein praxisorientierter Leitfaden zu Diagnostik und Therapie

Mast cell activation disease: A concise practical guide for diagnostic workup and therapeutic options
G. J. Molderings
1   Institut für Humangenetik, Universitätsklinikum Bonn
J. Homann
2   Abteilung für Innere Medizin, Gemeinschaftskrankenhaus Bonn
S. Brettner
3   Klinik für Onkologie, Hämatologie und Palliativmedizin, Kreiskrankenhaus Waldbröl
M. Raithel
4   Medizinische Klinik 1, Universitätsklinikum Erlangen
T. Frieling
5   Medizinische Klinik II, HELIOS Klinikum Krefeld
› Author Affiliations
Further Information

Publication History

04 November 2013

19 March 2014

Publication Date:
06 May 2014 (online)


In der vorliegenden Arbeit werden die klinischen Erscheinungsbilder der systemischen Mastzellaktivierungserkrankung, die pathogenetischen Zusammenhänge und diagnostischen Algorithmen sowie Therapieoptionen dargestellt. Dies soll den Leser in die Lage versetzen, eine systemische Mastzellaktivierungserkrankung zu erkennen, differenzialdiagnostisch einzugrenzen sowie eine Therapie empfehlen zu können. Bei chronischen unklaren multisystemischen Beschwerden sollte die systemische Mastzellaktivierungserkrankung frühzeitig in die differenzialdiagnostischen Überlegungen einbezogen werden. Mit spezifischen, wenig invasiven Untersuchungsverfahren kann in den meisten Fällen die Diagnose gesichert und eine erkrankungsgerechte, an der individuellen Symptomatik orientierte Therapie eingeleitet werden.


In the present paper clinical phenotypes, pathogenetic relationships, and diagnostic algorithms as well as therapeutic concepts of/for systemic mast cell activation disease are reviewed. The reader should be able to recognize and diagnose a systemic mast cell activation disease, as well as to counsel a personalized drug therapy. In the case of chronic multisystem polymorbidity systemic mast cell activation disease should be considered as a differential diagnosis at an early stage. In most cases, specific, little invasive investigations allow diagnosing the disease and, hence, an appropriate therapy can be initiated.

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