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1 results

  • Chapter 12 - Systemic Mastocytosis

  • Jon van der Walt, Attilio Orazi, Daniel A. Arber, University of Chicago
  • Book:
    Diagnostic Bone Marrow Haematopathology
    Published online:
    12 November 2020
    Print publication:
    21 January 2021, pp 181-199

  • Summary

    Mastocytosis is a diverse group of rare diseases due to a clonal proliferation of neoplastic mast cells that can involve a wide variety of organ systems. The two main categories of mastocytosis are cutaneous mastocytosis (CM) showing only skin involvement, and systemic mastocytosis (SM) with at least one extracutaneous organ involved. In many cases of SM, the bone marrow (BM) shows varying degrees of infiltration. Most cases of CM develop during childhood, while adult patients in their fifth and sixth decades tend to present with SM [1]. The clinical course can vary from spontaneous regression in young children with CM to a highly aggressive course primarily seen in adult patients. Even within the category of SM, the presentation can range from indolent to aggressive, and it is thus divided into five subcategories as outlined in the most recent edition of the 2016 World Health Organization (WHO) classification of mastocytosis (Table 12.1) [2]. Of note, mastocytosis is now considered a distinct clinicopathologic entity that is separate from other myeloproliferative neoplasms.