Evaluation and Classification of Mast Cell Disorders: A Difficult to Manage Pathology in Clinical Practice

Abstract

Mast cells are granulocytic immunomodulatory cells with an important role in physiologic and pathogenic processes due to their location at the junction between the internal and external environment and to their capacity to release a broad range of active mediators. Mast cells mediators have both pro-inflammatory and anti-inflammatory activities and are implicated in various and complex pathology. Mast cells disorders (MCDs) represent a heterogeneous pathology, with frequently difficult and challenging evaluation and diagnostic workup. MCDs can be primary, secondary to other diseases, or idiopathic. Increased research interest in this field was noted during the last decade and various classification criteria, as well as diagnostic and treatment recommendations, were proposed. The aim of this paper is to review the most recent published data on the classification and evaluation of mast cells disorders and to point out the main difficulties in diagnosing and managing these complex diseases in medical practice.