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Risk and management of patients with mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) pandemic: Expert opinions - 05/08/20

Doi : 10.1016/j.jaci.2020.06.009 
Peter Valent, MD a, , Cem Akin, MD, PhD b, Patrizia Bonadonna, MD c, Knut Brockow, MD d, Marek Niedoszytko, MD, PhD e, Boguslaw Nedoszytko, PhD f, Joseph H. Butterfield, MD g, Ivan Alvarez-Twose, MD, PhD h, Karl Sotlar, MD i, Juliana Schwaab, MD j, Mohamad Jawhar, MD j, Andreas Reiter, MD j, Mariana Castells, MD, PhD k, Wolfgang R. Sperr, MD a, Hanneke C. Kluin-Nelemans, MD, PhD l, Olivier Hermine, MD, PhD m, Jason Gotlib, MD, MS n, Roberta Zanotti, MD o, Sigurd Broesby-Olsen, MD p, Hans-Peter Horny, MD q, Massimo Triggiani, MD, PhD r, Frank Siebenhaar, MD s, Alberto Orfao, MD, PhD t, Dean D. Metcalfe, MD, MS u, Michel Arock, PharmD, PhD v, Karin Hartmann, MD w
a Department of Internal Medicine I, Division of Haematology and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria 
b Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich 
c Allergy Unit, Verona University Hospital, Verona, Italy 
d Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany 
e Department of Allergology, Medical University of Gdansk, Gdansk, Poland 
f Department of Dermatology, Medical University of Gdansk, Gdansk, Poland 
g Mayo Clinic, Division of Allergic Diseases, Rochester, Minn 
h Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain 
i Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria 
j Department of Hematology and Oncology, University Hospital Mannheim, Mannheim, Germany 
k Brigham and Women’s Hospital, Mastocytosis Center, Harvard Medical School, Boston, Mass 
l Department of Haematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 
m Imagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre national de référence des mastocytoses, Paris, France 
n Stanford Cancer Institute/Stanford University School of Medicine, Stanford, Calif 
o Department of Medicine, Section of Hematology, University of Verona, Verona, Italy 
p Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark 
q Institute of Pathology, Ludwig-Maximilian-University, Munich, Germany 
r Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy 
s Dermatological Allergology, Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt - Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany 
t Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL), IBSAL, CIBERONC and Department of Medicine, University of Salamanca, Salamanca, Spain 
u Department of Dermatology & Allergy, Charité Universitätsmedizin Berlin, Berlin, Germany 
v Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France 
w Division of Allergy, Department of Dermatology, and Department of Biomedicine, University of Basel, Basel, Switzerland 

Corresponding author: Peter Valent, MD, Department of Medicine I, Division of Hematology and Hemostaseology and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Waehringer Guertel 18-20, Vienna 1090, Austria.Department of Medicine IDivision of Hematology and Hemostaseology and Ludwig Boltzmann Institute for Hematology and OncologyMedical University of ViennaWaehringer Guertel 18-20Vienna1090Austria

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Abstract

The coronavirus disease 2019 (COVID-19) (caused by severe acute respiratory syndrome coronavirus 2) pandemic has massively distorted our health care systems and caused catastrophic consequences in our affected communities. The number of victims continues to increase, and patients at risk can only be protected to a degree, because the virulent state may be asymptomatic. Risk factors concerning COVID-19–induced morbidity and mortality include advanced age, an impaired immune system, cardiovascular or pulmonary diseases, obesity, diabetes mellitus, and cancer treated with chemotherapy. Here, we discuss the risk and impact of COVID-19 in patients with mastocytosis and mast cell activation syndromes. Because no published data are yet available, expert opinions are, by necessity, based on case experience and reports from patients. Although the overall risk to acquire the severe acute respiratory syndrome coronavirus 2 may not be elevated in mast cell disease, certain conditions may increase the risk of infected patients to develop severe COVID-19. These factors include certain comorbidities, mast cell activation–related events affecting the cardiovascular or bronchopulmonary system, and chemotherapy or immunosuppressive drugs. Therefore, such treatments should be carefully evaluated on a case-by-case basis during a COVID-19 infection. In contrast, other therapies, such as anti–mediator-type drugs, venom immunotherapy, or vitamin D, should be continued. Overall, patients with mast cell disorders should follow the general and local guidelines in the COVID-19 pandemic and advice from their medical provider.

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Key words : Mast cells, mastocytosis, tryptase, KIT D816V, coronavirus, COVID-19, SARS-CoV-2, mast cell activation syndrome

Abbreviations used : CM, COVID-19, HSCT, ISM, MC, MCAS, SARS-CoV-2, SM


Mappa


 P.V. was supported by the Austrian Science Fund (FWF; projects P32470-B and F4704-B20). J.G. is supported by the Charles and Ann Johnson Foundation. D.D.M. is supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases.
 Disclosure of potential conflict of interest: P. Valent received consultancy honoraria from Blueprint, Novartis, Deciphera, Celgene, and Incyte and a research grant from Pfizer. C. Akin received consultancy honoraria from Blueprint and Novartis and research grant from Blueprint and is an investigator in a clinical trial for Blueprint. M. Niedoszytko received consultancy honoraria from Novartis and AB Science and is an investigator in clinical trials for Novartis and AB Science. I. Alvarez-Twose received consultancy honoraria from Novartis and Blueprint. M. Jawhar received consultancy honoraria from Novartis. A. Reiter received consultancy honoraria from Novartis, Blueprint, and Deciphera and research support from Novartis. M. Castells is a principal investigator in a clinical trial for Blueprint. W. R. Sperr received consultancy honoraria from Thermofisher, AbbVie, Novartis, Pfizer, Incyte, Deciphera, Jazz, Teva, and Celgene. H. C. Kluin-Nelemans received consultancy honoraria from Novartis. O. Hermine received research funding from AB Science; is cofounder of AB Science; and received research funding (unrelated to this study) from Novartis, Inatherys, Celgene, BMS, and Takeda. J. Gotlib received consultancy honoraria from Novartis, Blueprint, and Deciphera and is an investigator in a clinical trial for Novartis, Blueprint, and Deciphera. R. Zanotti received consultancy (honoraria) from Novartis and Deciphera. S. Broesby-Olsen provided consultancy in a clinical trial for Blueprint and received consultancy honoraria from Novartis and ThermoFisher. H.-P. Horny received consultancy honoraria from Novartis, Deciphera, and Blueprint. M. Triggiani received consultancy honoraria from Novartis, Deciphera, and Blueprint and is an investigator in a clinical trial for Blueprint. F. Siebenhaar received consultancy honoraria and research support from Allakos, Blueprint, Novartis, and Uriach. A. Orfao received consultancy honoraria from Novartis. D. D. Metcalfe is an investigator in a clinical trial for Sanofi US Services. M. Arock received consultancy honoraria from Blueprint. K. Hartmann received consultancy honoraria from Allergopharma, ALK-Abelló, Blueprint, Deciphera, Menarini, and Novartis. The rest of the authors declare that they have no relevant conflicts of interest.


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