Current Challenges in Diagnosis and Management of Mast Cell Disorders
A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".
Deadline for manuscript submissions: closed (30 September 2024) | Viewed by 11556
Special Issue Editor
2. Department of Medicine Solna, Unit of Immunology and Allergy, Karolinska Institutet, SE-14186 Stockholm, Sweden
Interests: anaphylaxis; mastocytosis. MCAS; KIT D816V mutation; tryptase; total IgE; HAT
Special Issue Information
Dear Colleagues,
Mast cell disorders (MCDs) constitute a heterogeneous group of conditions that may be caused by an increased number of mast cells (MCs) and/or pathologically activated MCs. These disorders can vary in severity and comprise clonal (e.g., mastocytosis) and nonclonal (idiopathic) variants. Clonal MC disorders, characterized well by intrinsic MC defects, include KIT mutation D816V, and/or expression of aberrant MC receptors. The latter may cause a “hyperactive” state of MCs, leading to excessive release of mediators from MCs. It is essential to obtain accurate diagnosis due to the substantial overlapping in clinical presentation of these conditions.
Mastocytosis is a rare variant of clonal MC disorder and affects both children and adults. The World Health Organization (WHO) introduced a classification of mastocytosis which divides the condition into 2 main groups: cutaneous mastocytosis (CM), and systemic mastocytosis (SM) involving at least 1 extracutaneous organ. SM has been further classified into several subgroups including indolent systemic mastocytosis (ISM); systemic smoldering mastocytosis (SSM); systemic mastocytosis with an associated hematologic neoplasm (SM-AHN); aggressive systemic mastocytosis (ASM); mast cell leukemia (MCL); and mast cell sarcoma (MCS).
Mast cell activation syndrome (MCAS) is another special variant of MCDs, characterized by severe, recurrent, and episodic symptoms caused by systemic MC mediator release. Patients with MCAS often present with symptoms of anaphylaxis. The diagnosis of MCAS is based on three diagnostic criteria: (1) the typical clinical symptoms consistent with severe, acute MC activation; (2) an event-related increase in serum tryptase above the individual's baseline tryptase (sBT), according to the formula: sBT + 20% of sBT (= 120% of sBT = sBT × 1.2) plus 2 ng/mL; and (3) the response to drugs directed against MC activation or the production or effects of MC mediators.
In this Special Issue, we invite original research articles and reviews focused on the latest developments in mastocytosis and other related MCDs. We are particularly interested in the novel biomarkers that could be used to predict patients with different variant of mast cell disorders.
Dr. Theo Gulen
Guest Editor
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Keywords
- mastocytosis
- mast cell
- mast cell activation syndromes
- anaphylaxis
- novel biomarkers
- serum tryptase
- KIT D816V mutation
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