Mast Cell Diseases and Drug Hypersensitivity Reactions

Abstract

Mastocytosis is a clonal disorder characterized by the proliferation and accumulation of mast cells (MCs) in different tissues, with a preferential localization in skin and bone marrow (BM). Patients with cutaneous and systemic mastocytosis could present symptoms, including immediate and severe hypersensitivity reactions, related to the increase of MC releasability and to the excess of mast cell in the tissues. Mastocytosis in adults is associated with a history of anaphylaxis in 22–49%. Hymenoptera stings represent the more frequent elicitor of anaphylaxis, sometimes fatal, in mastocytosis patients. Moreover, anaphylaxis after intake of drugs such as non-steroidal anti-inflammatory drugs, opioids and drugs in the perioperative setting is occasionally described in mastocytosis. However, data on the frequency of drug hypersensitivity in mastocytosis and vice versa are scarce and evidence for an association appears to be limited. Nevertheless, clonal MC disorders should be ruled out in cases of severe anaphylaxis based on the evaluation of basal serum tryptase determination, physical examination for cutaneous Mastocytosis lesions, and clinical characteristics of anaphylactic reaction. In this paper, we focused on the most relevant evidence about the association between mastocytosis and hypersensitivity to some classes of drugs. In addition, we tried to give recommendations, based on available literature data, about the use of these drugs in patients with diagnosed mastocytosis.