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.
Highlights
BackgroundMast cells (MCs) are complex and interesting cells, even after 140 years from their discovery, by Paul Ehrlich, who is considered the founder of modern immunology [1]
Mast cells are morphologically defined by numerous electron-dense cytoplasmic granules, which contain a plethora of active mediators, such as biogenic amines, serines and other proteases, lysosomal enzymes, and proteoglycans [3]
The aim of this paper is to review the most recent published data on the classification and evaluation of mast cell disorders and to point out the main difficulties in diagnosing and managing this complex group of disorders in medical practice
Summary
Mast cells (MCs) are complex and interesting cells, even after 140 years from their discovery, by Paul Ehrlich, who is considered the founder of modern immunology [1]. Confirmation of the systemic mastocytosis diagnosis is based on expert recommendations and guidelines criteria but may be difficult in cases with incomplete diagnostic criteria, requiring allergist expertise and multidisciplinary evaluation [21] This is a very heterogeneous group of disorders, with unknown prevalence, characterized by episodic spontaneous mast cell activation and degranulation and nonspecific symptoms, with variable severity but without MC hyperplasia (non-clonal proliferation) [22]. Most authors consider that the MCAS diagnosis is attributed to patients with severe and episodic symptoms affecting many organ systems, suggesting anaphylaxis, rather than to those with nonspecific symptoms, such as recurrent flushing and/or gastrointestinal symptoms This is a particular variant of indolent systemic mastocytosis without skin involvement, with normal or near-normal serum tryptase level, leading to a more difficult and delayed diagnosis compared to other forms. Recent inquiry of MCDs patient groups from 12 countries referring to patients' concerns and expectations from the scientific community revealed the need for better education and knowledge of physicians, increased awareness, better access to specialized centers, improved diagnostic criteria, and better treatments [37]
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