What are the WHO diagnostic criteria for systemic mast cell disease?

Updated: Jun 07, 2018
  • Author: Jacquiline Habashy, DO, MSc; Chief Editor: Dirk M Elston, MD  more...
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Answer

WHO major criteria for the diagnosis of systemic mast cell disease include the following [24] :

  • Multifocal dense infiltrates of mast cells (>15 close to each other) observed in bone marrow biopsy specimens and/or mast cells stained for tryptase in biopsy specimens from other extracutaneous organs.

WHO minor major criteria for the diagnosis of systemic mast cell disease include the following [24] :

  • Mast cells in bone marrow, or other extracutaneous organs show abnormal (spindling) morphology (>25%) or the presence of greater than 25% atypical mast cells in bone marrow aspirates

  • Demonstration of c-kit point mutation on codon 816 in bone marrow, blood, or other extracutaneous organs

  • CD117 (c-kit receptor)–positive cells, also positive for CD2 and/or CD25

  • Serum tryptase values greater than 20 ng/mL [2]


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