Aktuelle Dermatologie 2021; 47(08/09): 376-379
DOI: 10.1055/a-1539-1453
Übersicht

Mastozytose – eine kurze Übersicht

Mastocytosis – A Short Review
Hans-Peter Horny
Europäisches Referenzzentrum für Mastozytose im ECNM, Institut für Pathologie der LMU, München
,
Peter Valent
Europäisches Referenzzentrum für Mastozytose im ECNM, Institut für Pathologie der LMU, München
› Author Affiliations

Zusammenfassung

Die Mastozytose ist eine ungewöhnliche Erkrankung des hämatopoetischen Gewebes (Knochenmark) mit heterogenem klinischem Erscheinungsbild und dementsprechend ganz unterschiedlicher Prognose. Prinzipiell wird eine rein kutane Mastozytose (fast immer bei Kindern) von einer systemischen Mastozytose (bei Erwachsenen) mit fakultativem, gleichwohl häufigem Hautbefall unterschieden. Im Zentrum der Diagnose steht der Histopathologe mit Nachweis einer Zunahme atypischer Mastzellen im Gewebeschnitt, häufig unter Ausbildung kompakter Infiltrate.

Abstract

Mastocytosis comprises a group of very heterogeneous subvariants with markedly differing prognosis. The histopathological key feature is an increase in atypical mast cells often forming compact infiltrates. Therefore, the pathologist plays a crucial role in the diagnostic approach to the disease.



Publication History

Article published online:
19 August 2021

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