A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders
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 acc...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/22/3/1454 |
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| author | Jan Romantowski Aleksandra Górska Marek Niedoszytko Theo Gulen Marta Gruchała-Niedoszytko Bogusław Nedoszytko Magdalena Lange Knut Brockow Michel Arock Cem Akin Peter Valent |
| author_facet | Jan Romantowski Aleksandra Górska Marek Niedoszytko Theo Gulen Marta Gruchała-Niedoszytko Bogusław Nedoszytko Magdalena Lange Knut Brockow Michel Arock Cem Akin Peter Valent |
| author_sort | Jan Romantowski |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T06:11:47Z |
| format | Article |
| id | doaj.art-a0f464358ebd4a83ab5fa18e099a594a |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-09T06:11:47Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-a0f464358ebd4a83ab5fa18e099a594a2023-12-03T11:58:11ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-02-01223145410.3390/ijms22031454A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell DisordersJan Romantowski0Aleksandra Górska1Marek Niedoszytko2Theo Gulen3Marta Gruchała-Niedoszytko4Bogusław Nedoszytko5Magdalena Lange6Knut Brockow7Michel Arock8Cem Akin9Peter Valent10Department of Allergology, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Allergology, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Allergology, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Respiratory Medicine and Allergy, Karolinska University Hospital, 14186 Huddinge, SwedenDepartment of Clinical Nutrition, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Dermatology, Venerology and Allergology, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Dermatology, Venerology and Allergology, Medical University of Gdansk, 80-211 Gdańsk, PolandDepartment of Dermatology and Allergy, School of Medicine, Technical University of Munich, D-80802 Munich, GermanyDepartment of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), 75005 Paris, FranceDivision of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI 48106, USADepartment of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, 1090 Vienna, AustriaPrimary 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.https://www.mdpi.com/1422-0067/22/3/1454mast cellanaphylaxismastocytosishypersensitivityMCAS |
| spellingShingle | Jan Romantowski Aleksandra Górska Marek Niedoszytko Theo Gulen Marta Gruchała-Niedoszytko Bogusław Nedoszytko Magdalena Lange Knut Brockow Michel Arock Cem Akin Peter Valent A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders International Journal of Molecular Sciences mast cell anaphylaxis mastocytosis hypersensitivity MCAS |
| title | A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders |
| title_full | A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders |
| title_fullStr | A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders |
| title_full_unstemmed | A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders |
| title_short | A Challenge for Allergologist: Application of Allergy Diagnostic Methods in Mast Cell Disorders |
| title_sort | challenge for allergologist application of allergy diagnostic methods in mast cell disorders |
| topic | mast cell anaphylaxis mastocytosis hypersensitivity MCAS |
| url | https://www.mdpi.com/1422-0067/22/3/1454 |
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