Bone Marrow Expression of Mast Cell Disorders

Immunol Allergy Clin North Am. 2018 Aug;38(3):379-395. doi: 10.1016/j.iac.2018.04.012.

Abstract

Mast cell disorders comprise a heterogeneous group of rare diseases, the diagnosis of which still remains a challenge. Bone marrow analysis constitutes the most appropriate site for screening systemic involvement in mastocytosis. Morphologic, immunohistochemical, flow cytometric immunophenotyping, and molecular studies should be routinely performed for diagnostic/prognostic purposes in experienced reference centers during the diagnostic workup in suspected systemic mastocytosis. The authors review the most relevant characteristics of bone marrow expression of mast cell disorders as well as the different methodological approaches to be applied to perform an objective and reproducible diagnosis and classification of mastocytosis and other mast cell disorders.

Keywords: Bone marrow; Cell purification; Diagnosis; Flow cytometry; KIT mutation; Mast cells; Mastocytosis; Prognosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Biomarkers / metabolism
  • Bone Marrow / metabolism
  • Bone Marrow / pathology*
  • Bone Marrow Diseases / diagnosis
  • Bone Marrow Diseases / genetics
  • Bone Marrow Diseases / immunology*
  • Clone Cells
  • Humans
  • Immunohistochemistry / methods
  • Mast Cells / physiology*
  • Mastocytosis / diagnosis
  • Mastocytosis / genetics
  • Mastocytosis / immunology*
  • Mutation / genetics*
  • Proto-Oncogene Proteins c-kit / genetics*
  • Proto-Oncogene Proteins c-kit / metabolism

Substances

  • Biomarkers
  • KIT protein, human
  • Proto-Oncogene Proteins c-kit