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Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy

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A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (28 February 2021) | Viewed by 43634

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Dr. Bogusław Nedoszytko

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Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Poland
Interests: molecular basis of mastocytosis; psoriasis; atopic dermatitis; epigenetic changes in psoriasis and mastocytosis; chromosomal changes in melanoma; sarcomas and T-cell lymphomas
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Peter Valent

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1. Division of Hematology & Hemostaseology, Department of Internal Medicine, Medical University of Vienna, Vienna, Austria
2. Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria
Interests: basophils and tissue mast cells; Pathophysiology of AML; CML; MDS; MCAS and mastocytosis; eosinophilic granulocytes and eosinophil leukemia; oncogene dependent signaling in leukemia cells; identification of new therapeutic targets in neoplastic cells; characterization and eradication of leukemic stem cells; synergistic effects of new anti-leukemic drugs

Prof. Dr. Marek Niedoszytko

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Department of Allergology Medical University of Gdańsk, Poland
Interests: molecular basis of mastocytosis; MCAS; pulmonary diseases; allergy; anaphylaxis; immunotherapy; epigenetic changes in mastocytosis; gene expression in mastocytosis

Special Issue Information

Dear Colleagues,

Mast cells (MC) are multifunctional cells regulating innate and adaptative immune system. MC participate in detection of harmful pathogens like viruses, bacteria, parasites and toxins, play the role in wound healing, cancer and tumor progression. Inappropriate, recurrent mast cell activation and secretion MC-derived mediators plays an essential role in many human diseases: allergy, asthma, allergic rhinitis, urticaria, anaphylaxis, atopic dermatitis, mastocytosis (MCT) and mast cell activation syndrome (MCAS).

Mastocytosis is a heterogeneous group of myelodysplastic diseases with abnormal clonal MC proliferation, activation and accumulation of MC in the skin, bone marrow and/or other visceral organs. The diagnosis is based on the WHO criteria, in which the tryptase level, histopathological and immunophenotypic (CD2/CD25) assessment of MCs and detection of D816V somatic mutation of KIT gene are crucial. The disease is divided into 7 variants: cutaneous mastocytosis, indolent systemic mastocytosis (ISM), SM with an associated clonal haematological non‐MC‐lineage disease (SM‐AHNMD), aggressive SM (ASM), MC leukaemia (MCL), MC sarcoma (MCS), and extracutaneous mastocytoma.

MCAS can be diagnosed based three main criteria: a) typical symptoms symptoms caused by increased degranulation of MCs, b) serum tryptase elevation and c) response to anti-mediator treatment. The disease is classified as primary, secondary, and idiopathic. In the primary MCAS, monoclonal abnormal MC proliferation, with KIT D816V mutation and /or CD25⁺MC in bone marrow biopsy are observed. Secondary MCAS is caused by allergy or another underlying disease, with MC negative for D816V KIT and CD2/CD25.

Symptoms of MCAS and SM can be managed by blockade of mediator receptors (H1 and H2 antihistamines), leukotriene receptor blockage, inhibition of mediator synthesis or release, anti-IgE therapy, or a combination of these medications. Acute episodes of MC activation require epinephrine. Patients with SM or primary MCAS may need a cytoreductive therapy to prevent severe symptoms including anaphylaxis.

In this special Issue, we will publish research papers, up-to-date review articles, and commentaries are all welcome.

Dr. Bogusław Nedoszytko
Prof. Dr. Peter Valent
Prof. Dr. Marek Niedoszytko
Guest Editors

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Keywords

Mast cells
Mastocytosis
Mast cell activation syndrome
Urticaria pigmentosa
Anaphylaxis
Tryptase
CD2/CD25
Activating mutations of KIT receptor gene
Familial alpha hypertryptasemia
Epigenetics

Published Papers (10 papers)

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Editorial

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2 pages, 162 KiB

Open AccessEditorial

Mastocytosis, MCAS, and Related Disorders—Diagnosis, Classification, and Therapy

by Marek Niedoszytko, Peter Valent and Bogusław Nedoszytko

Int. J. Mol. Sci. 2021, 22(9), 5024; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22095024 - 10 May 2021

Cited by 2 | Viewed by 2117

Abstract

Mastocytosis is a heterogeneous group of hematologic neoplasms defined by an accumulation of neoplastic mast cells (MC) in the skin, bone marrow, and other visceral organs [...] Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

Research

Jump to: Editorial, Review

17 pages, 647 KiB

Open AccessArticle

Results from a Genome-Wide Association Study (GWAS) in Mastocytosis Reveal New Gene Polymorphisms Associated with WHO Subgroups

by Bogusław Nedoszytko, Marta Sobalska-Kwapis, Dominik Strapagiel, Magdalena Lange, Aleksandra Górska, Joanne N. G. Oude Elberink, Jasper van Doormaal, Marcin Słomka, Leszek Kalinowski, Marta Gruchała-Niedoszytko, Roman J. Nowicki, Peter Valent and Marek Niedoszytko

Int. J. Mol. Sci. 2020, 21(15), 5506; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21155506 - 31 Jul 2020

Cited by 10 | Viewed by 3569

Abstract

Mastocytosis is rare disease in which genetic predisposition is not fully understood. The aim of this study was to analyze associations between mastocytosis and single nucleotide polymorphisms (SNPs) by a genome-wide association study (GWAS) approach. A total of 234 patients were enrolled in our study, including 141 with cutaneous mastocytosis (CM; 78 children and 63 adults) and 93 with systemic mastocytosis (SM, all adults). The control group consisted of 5606 healthy individuals. DNA samples from saliva or blood were genotyped for 551 945 variants using DNA microarrays. The prevalence of certain SNPs was found to vary substantially when comparing patients and healthy controls: rs10838094 of 5OR51Q1 was less frequently detected in CM and SM patients (OR = 0.2071, p = 2.21 × 10⁻²⁹), rs80138802 in ABCA2 (OR = 5.739, p = 1.98 × 10⁻²⁸), and rs11845537 in OTX2-AS1 (rs11845537, OR = 6.587, p = 6.16 × 10⁻¹⁷) were more frequently detected in CM in children and adults. Additionally, we found that rs2279343 in CYP2B6 and rs7601511 in RPTN are less prevalent in CM compared to controls. We identified a number of hitherto unknown associations between certain SNPs and CM and/or SM. Whether these associations are clinically relevant concerning diagnosis, prognosis, or prevention remains to be determined in future studies. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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Review

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15 pages, 500 KiB

Open AccessReview

Response Criteria in Advanced Systemic Mastocytosis: Evolution in the Era of KIT Inhibitors

by William Shomali and Jason Gotlib

Int. J. Mol. Sci. 2021, 22(6), 2983; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22062983 - 15 Mar 2021

Cited by 24 | Viewed by 3780

Abstract

Systemic mastocytosis (SM) is a rare clonal hematologic neoplasm, driven, in almost all cases, by the activating KIT D816V mutation that leads to the growth and accumulation of neoplastic mast cells. While patients with advanced forms of SM have a poor prognosis, the introduction of KIT inhibitors (e.g., midostaurin, and avapritinib) has changed their outlook. Because of the heterogenous nature of advanced SM (advSM), successive iterations of response criteria have tried to capture different dimensions of the disease, including measures of mast cell burden (percentage of bone marrow mast cells and serum tryptase level), and mast cell-related organ damage (referred to as C findings). Historically, response criteria have been anchored to reversion of one or more organ damage finding(s) as a minimal criterion for response. This is a central principle of the Valent criteria, Mayo criteria, and International Working Group-Myeloproliferative Neoplasms Research and Treatment and European Competence Network on Mastocytosis (IWG-MRT-ECNM) consensus criteria. Irrespective of the response criteria, an ever-present challenge is how to apply response criteria in patients with SM and an associated hematologic neoplasm, where the presence of both diseases complicates assignment of organ damage and adjudication of response. In the context of trials with the selective KIT D816V inhibitor avapritinib, pure pathologic response (PPR) criteria, which rely solely on measures of mast cell burden and exclude consideration of organ damage findings, are being explored as more robust surrogate of overall survival. In addition, the finding that avapritinib can elicit complete molecular responses of KIT D816V allele burden, establishes a new benchmark for advSM and motivates the inclusion of definitions for molecular response in future criteria. Herein, we also outline how the concept of PPR can inform a proposal for new response criteria which use a tiered evaluation of pathologic, molecular, and clinical responses. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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13 pages, 299 KiB

Open AccessReview

Epigenetic Changes in Neoplastic Mast Cells and Potential Impact in Mastocytosis

by Edyta Reszka, Ewa Jabłońska, Edyta Wieczorek, Peter Valent, Michel Arock, Gunnar Nilsson, Bogusław Nedoszytko and Marek Niedoszytko

Int. J. Mol. Sci. 2021, 22(6), 2964; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22062964 - 15 Mar 2021

Cited by 7 | Viewed by 1946

Abstract

Systemic mastocytosis (SM) is a hematologic neoplasm with abnormal accumulation of mast cells in various organ systems such as the bone marrow, other visceral organs and skin. So far, only little is known about epigenetic changes contributing to the pathogenesis of SM. In the current article, we provide an overview of epigenetic changes that may occur and be relevant to mastocytosis, including mutations in genes involved in epigenetic processes, such as TET2, DNMT3A and ASXL1, and global and gene-specific methylation patterns in neoplastic cells. Moreover, we discuss methylation-specific pathways and other epigenetic events that may trigger disease progression in mast cell neoplasms. Finally, we discuss epigenetic targets and the effects of epigenetic drugs, such as demethylating agents and BET-targeting drugs, on growth and viability of neoplastic mast cells. The definitive impact of these targets and the efficacy of epigenetic therapies in advanced SM need to be explored in future preclinical studies and clinical trials. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

14 pages, 727 KiB

Open AccessReview

by Knut Brockow, Katarzyna Plata-Nazar, Magdalena Lange, Bogusław Nedoszytko, Marek Niedoszytko and Peter Valent

Int. J. Mol. Sci. 2021, 22(5), 2684; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22052684 - 07 Mar 2021

Cited by 23 | Viewed by 3689

Abstract

Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier’s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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23 pages, 7701 KiB

Open AccessReview

Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021

by Magdalena Lange, Karin Hartmann, Melody C. Carter, Frank Siebenhaar, Ivan Alvarez-Twose, Inés Torrado, Knut Brockow, Joanna Renke, Ninela Irga-Jaworska, Katarzyna Plata-Nazar, Hanna Ługowska-Umer, Justyna Czarny, Anna Belloni Fortina, Francesca Caroppo, Roman J. Nowicki, Bogusław Nedoszytko, Marek Niedoszytko and Peter Valent

Int. J. Mol. Sci. 2021, 22(5), 2586; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22052586 - 04 Mar 2021

Cited by 35 | Viewed by 5173

Abstract

Pediatric mastocytosis is a heterogeneous disease characterized by accumulation of mast cells in the skin and less frequently in other organs. Somatic or germline mutations in the KIT proto-oncogene are detected in most patients. Cutaneous mastocytosis is the most common form of the disease in children. In the majority of cases, skin lesions regress spontaneously around puberty. However, in few patients, mastocytosis is not a self-limiting disease, but persists into adulthood and can show signs of systemic involvement, especially when skin lesions are small-sized and monomorphic. Children with mastocytosis often suffer from mast cell mediator-related symptoms. Severe hypersensitivity reactions can also occur, mostly in patients with extensive skin lesions and blistering. In a substantial number of these cases, the triggering factor of anaphylaxis remains unidentified. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers, treatment with H1 and H2 histamine receptor blockers, and equipment of patients and their families with epinephrine auto-injectors for use in severe anaphylactic reactions. Advanced systemic mastocytosis occurs occasionally. All children with mastocytosis require follow-up examinations. A bone marrow investigation is performed when advanced systemic mastocytosis is suspected and has an impact on therapy or when cutaneous disease persists into adulthood. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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22 pages, 724 KiB

Open AccessReview

Genetic Regulation of Tryptase Production and Clinical Impact: Hereditary Alpha Tryptasemia, Mastocytosis and Beyond

by Bettina Sprinzl, Georg Greiner, Goekhan Uyanik, Michel Arock, Torsten Haferlach, Wolfgang R. Sperr, Peter Valent and Gregor Hoermann

Int. J. Mol. Sci. 2021, 22(5), 2458; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22052458 - 28 Feb 2021

Cited by 24 | Viewed by 4692

Abstract

Tryptase is a serine protease that is predominantly produced by tissue mast cells (MCs) and stored in secretory granules together with other pre-formed mediators. MC activation, degranulation and mediator release contribute to various immunological processes, but also to several specific diseases, such as IgE-dependent allergies and clonal MC disorders. Biologically active tryptase tetramers primarily derive from the two genes TPSB2 (encoding β-tryptase) and TPSAB1 (encoding either α- or β-tryptase). Based on the most common gene copy numbers, three genotypes, 0α:4β, 1α:3β and 2α:2β, were defined as “canonical”. About 4–6% of the general population carry germline TPSAB1-α copy number gains (2α:3β, 3α:2β or more α-extra-copies), resulting in elevated basal serum tryptase levels. This condition has recently been termed hereditary alpha tryptasemia (HαT). Although many carriers of HαT appear to be asymptomatic, a number of more or less specific symptoms have been associated with HαT. Recent studies have revealed a significantly higher HαT prevalence in patients with systemic mastocytosis (SM) and an association with concomitant severe Hymenoptera venom-induced anaphylaxis. Moreover, HαT seems to be more common in idiopathic anaphylaxis and MC activation syndromes (MCAS). Therefore, TPSAB1 genotyping should be included in the diagnostic algorithm in patients with symptomatic SM, severe anaphylaxis or MCAS. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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14 pages, 347 KiB

Open AccessReview

A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders

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

Int. J. Mol. Sci. 2021, 22(3), 1454; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22031454 - 01 Feb 2021

Cited by 7 | Viewed by 2972

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. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

18 pages, 848 KiB

Open AccessReview

Clinical Impact of Inherited and Acquired Genetic Variants in Mastocytosis

by Boguslaw Nedoszytko, Michel Arock, Jonathan J. Lyons, Guillaume Bachelot, Lawrence B. Schwartz, Andreas Reiter, Mohamad Jawhar, Juliana Schwaab, Magdalena Lange, Georg Greiner, Gregor Hoermann, Marek Niedoszytko, Dean D. Metcalfe and Peter Valent

Int. J. Mol. Sci. 2021, 22(1), 411; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22010411 - 02 Jan 2021

Cited by 20 | Viewed by 4571

Abstract

Mastocytosis is a rare and complex disease characterized by expansion of clonal mast cells (MC) in skin and/or various internal organ systems. Involvement of internal organs leads to the diagnosis of systemic mastocytosis (SM). The WHO classification divides SM into indolent SM, smoldering SM and advanced SM variants, including SM with an associated hematologic neoplasm, aggressive SM, and MC leukemia. Historically, genetic analysis of individuals with pure cutaneous mastocytosis (CM) and SM have focused primarily on cohort studies of inherited single nucleotide variants and acquired pathogenic variants. The most prevalent pathogenic variant (mutation) in patients with SM is KIT p.D816V, which is detectable in most adult patients. Other somatic mutations have also been identified—especially in advanced SM—in TET2, SRSF2, ASXL1, RUNX1, CBL and JAK2, and shown to impact clinical and cellular phenotypes. Although only small patient cohorts have been analyzed, disease associations have also been identified in several germline variants within genes encoding certain cytokines or their receptors (IL13, IL6, IL6R, IL31, IL4R) and toll-like receptors. More recently, an increased prevalence of hereditary alpha-tryptasemia (HαT) caused by increased TPSAB1 copy number encoding alpha-tryptase has been described in patients with SM. Whereas HαT is found in 3–6% of general Western populations, it is identified in up to 17% of patients with SM. In the current manuscript we review the prevalence, functional role and clinical impact of various germline and somatic genetic variants in patients with mastocytosis. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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14 pages, 1367 KiB

Open AccessReview

Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine

by Peter Valent, Cem Akin, Boguslaw Nedoszytko, Patrizia Bonadonna, Karin Hartmann, Marek Niedoszytko, Knut Brockow, Frank Siebenhaar, Massimo Triggiani, Michel Arock, Jan Romantowski, Aleksandra Górska, Lawrence B. Schwartz and Dean D. Metcalfe

Int. J. Mol. Sci. 2020, 21(23), 9030; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21239030 - 27 Nov 2020

Cited by 56 | Viewed by 8554

Abstract

Mast cell activation (MCA) is seen in a variety of clinical contexts and pathologies, including IgE-dependent allergic inflammation, other immunologic and inflammatory reactions, primary mast cell (MC) disorders, and hereditary alpha tryptasemia (HAT). MCA-related symptoms range from mild to severe to life-threatening. The severity of MCA-related symptoms depends on a number of factors, including genetic predisposition, the number and releasability of MCs, organs affected, and the type and consequences of comorbid conditions. In severe systemic reactions, MCA is demonstrable by a substantial increase of basal serum tryptase levels above the individual’s baseline. When, in addition, the symptoms are recurrent, involve more than one organ system, and are responsive to therapy with MC-stabilizing or mediator-targeting drugs, the consensus criteria for the diagnosis of MCA syndrome (MCAS) are met. Based on the etiology of MCA, patients can further be classified as having i) primary MCAS where KIT-mutated, clonal, MCs are detected; ii) secondary MCAS where an underlying IgE-dependent allergy or other reactive MCA-triggering pathology is found; or iii) idiopathic MCAS, where neither a triggering reactive state nor KIT-mutated MCs are identified. Most severe MCA events occur in combined forms of MCAS, where KIT-mutated MCs, IgE-dependent allergies and sometimes HAT are detected. These patients may suffer from life-threatening anaphylaxis and are candidates for combined treatment with various types of drugs, including IgE-blocking antibodies, anti-mediator-type drugs and MC-targeting therapy. In conclusion, detailed knowledge about the etiology, underlying pathologies and co-morbidities is important to establish the diagnosis and develop an optimal management plan for MCAS, following the principles of personalized medicine. Full article

(This article belongs to the Special Issue Mastocytosis, MCAS, and Related Disorders – Diagnosis, Classification and Therapy)

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