Mastocytosis: Lessons Learned from the 2019 Society for Hematopathology/European Association for Haematopathology Workshop

Alexandar Tzankov, Eric Duncavage, Fiona E. Craig, Katalin Kelemen, Rebecca L. King, Attilio Orazi, Leticia Quintanilla-Martinez, Kaaren K. Reichard, Lisa M. Rimsza, Sa A. Wang, Hans Peter Horny, Tracy I. George

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Objectives: The 2019 Workshop of the Society for Hematopathology/European Association for Haematopathology received and reviewed cases covering the spectrum of mastocytosis and related diseases, including morphologic mimics, focusing on recent updates and relevant findings for pathologists. Methods: The workshop panel reviewed 99 cases of cutaneous and systemic mastocytosis (SM) and SM and associated hematologic neoplasms (SM-AHN). Results: Despite a common theme of KIT mutation (particularly D816V), mastocytosis is a heterogeneous neoplasm with a wide variety of presentations. This spectrum, including rare subtypes and extramedullary organ involvement, is discussed and illustrated by representative cases. Conclusions: In the age of targeted treatment aimed at KIT, the accurate diagnosis and classification of mastocytosis has major implications for therapy and further interventions. Understanding the clinical, pathologic, and genetic findings of mastocytosis is crucial for selecting the proper tests to perform and subsequent arrival at a correct diagnosis in this rare disease.

Original languageEnglish (US)
Pages (from-to)239-266
Number of pages28
JournalAmerican journal of clinical pathology
Volume155
Issue number2
DOIs
StatePublished - Feb 1 2021

Keywords

  • D816V
  • KIT
  • Mast cell
  • Mastocytosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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