A challenge for allergologist: Application of allergy diagnostic methods in mast cell disorders

Jan Romantowski, Aleksandra Górska, Marek Niedoszytko, Theo Gulen, Marta Gruchała-Niedoszytko, Bogusław Nedoszytko, Magdalena Lange, Knut Brockow, Michel Arock, Cem Akin, Peter Valent

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

Abstract

Primary and secondary mast cell activation syndromes (MCAS) can occur in patients with mastocytosis. During the past few years our knowledge about the pathogenesis and disease-triggering mechanisms in MCAS and mastocytosis have increased substantially. Whereas mastocytosis is characterized by an accumulation of neoplastic (clonal) mast cells (MC) in various organ systems, MCAS is defined by a massive and systemic activation of these cells. Mast cells are crucial effector cells in allergic diseases, thus their elevated number and activation can cause severe anaphylactic reactions and MCAS in patients with mastocytosis. However, these cells may also degranulate spontaneously or degranulate in response to non-allergic triggers leading to clinical symptoms. In mastocytosis patients, such symptoms may lead to the diagnosis of a primary MCAS. The diagnosis of a concomitant allergy in mastocytosis patients is challenging. In these patients, a mixed form (primary and secondary) of MCAS may be diagnosed. These patients may also suffer from life-threatening anaphylactic reactions when exposed to allergens. In these cases, the possibility of severe side effects of in vivo provocations can sometimes also limit diagnostic evaluations. In the current article, we discuss the diagnosis and management of patients suffering from mastocytosis and concomitant MCAS, with special emphasis on novel diagnostic tests and management, including allergen microarrays, recombinant allergen analysis, basophil activation tests, optimal prophylaxis, and specific therapies.

Original languageEnglish
Article number1454
Pages (from-to)1-14
Number of pages14
JournalInternational Journal of Molecular Sciences
Volume22
Issue number3
DOIs
StatePublished - 2 Feb 2021
Externally publishedYes

Keywords

  • Anaphylaxis
  • Hypersensitivity
  • MCAS
  • Mast cell
  • Mastocytosis

Fingerprint

Dive into the research topics of 'A challenge for allergologist: Application of allergy diagnostic methods in mast cell disorders'. Together they form a unique fingerprint.

Cite this