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Zugrunde liegt eine klonale Mastzellvermehrung, meist in der Haut oder dem Knochenmark, seltener im Gastrointestinaltrakt oder anderen Organen. In der Regel haben Mastozytosen eine gute Prognose quoad vitam. Seltene Formen, wie die Mastzellleuk\u00e4mie, die aggressive Mastozytose und das extrem rare Mastzellsarkom erfordern eine zytoreduktive Therapie. 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