Unfortunately, due to the health of our team, Mast Cell Hope has closed down in December of 2023. We will be maintaining this site for educational purposes for as long as we can. Visit our education page for learn about mast cell disease.

Learning Resources

TREATMENT OF MAST CELL DISEASE

There is currently no cure for mast cell disease. Treatment is aimed at reducing the occurrence of episodes and reducing the severity of symptoms.

Prophylactic Avoidance of Identified Triggers

The first step to managing mast cell disease is to adopt a prophylactic approach to all identified known and potential triggers.1

Prophylactic Treatment with Medication

Antihistamine Medications (Histamine Blockers)

H1 Blocker Antihistamines

H1 antihistamines help treat pruritus (itchy skin), flushing of the skin, and help achieve general mast cell stability, therefore helping to treat a wide range of symptoms associated with mast cell instability.2,3

1st Generation H1 Blockers
  • Chlorpheniramine (Chlortrimeton®)
  • Diphenhydramine (Benadryl®)
  • Doxepin hydrochloride (Doxepin®, Sinequan®)
  • Hydroxyzine hydrochloride (Atarax®)
  • Oral ketotifen
2nd Generation H1 Blockers
  • Cetirizine (Zyrtec®)
  • Desloratadine (Clarinex®)
  • Fexofenadine (Allegra®)
  • Levocetirizine (Xyzal®) 3rd Generation
  • Loratadine (Claritin®)

Mast Cell Hope tip: Second or third generation H1 blockers are generally trialed before first generation H1 blockers. Benadryl® should not be used unless the patient has failed all other H1 blockers. In patients who respond to other H1 blockers, Benadryl® should be used as a rescue medication.

H2 Blocker Antihistamines

HR2 blocker antihistamines help manage symptoms of the gastrointestinal tract, such as cramps, constipation, and diarrhea.2

  • Cimetidine (Tagamet®)
  • Famotidine (Pepcid®)

Mast Cell Stabilizers

Mast cell stabilizers are most often prescribed in combination with histamine blockers and help treat a wide range of symptoms. Patients may sometimes need to take more than one mast cell stabilizer, depending on their symptoms. For example, oral cromolyn sodium is used to treat gastrointestinal issues, while ketotifen is used to treat general mast cell symptoms.4,5

  • Bioflavonoids such as quercetin and luteolin6,7
  • Oral cromolyn sodium (Gastrocrom®)4,8
  • Topical cromolyn sodium9
  • Oral ketotifen5,10
  • Vitamin C11
  • Vitamin D12

Leukotriene Inhibitors

Leukotriene inhibitors are most often prescribed in combination with histamine blockers. Leukotriene inhibitors help treat asthma and respiratory symptoms in addition to psoriasis and dermatitis.13

  • Montelukast (Singulair ®)
  • Zafirlukast (Accolate®)
  • Zileuton (Zyflo®/Zyflo CR®)

Aspirin Therapy

Aspirin may be prescribed in small doses in combination with histamine blockers to help manage symptoms, such as flushing of the skin and symptoms associated with the excess release of prostaglandin (PG) D2.14,15

Do not self-treat/medicate with aspirin! Aspirin, while a miracle treatment for some, may cause anaphylaxis for others.

While aspirin is available over-the-counter, aspirin therapy should always be initiated and managed under the direct supervision of a physician.

Since long-term use of aspirin is associated with a risk of gastrointestinal bleeding, aspirin therapy is not recommended for patients with conditions of the GI tract or with an increased risk of ulcerative GI conditions.2,16

Anti-IgE Therapy

Anti-IgE therapy helps with overall mast cell stability and therefore a wide range of symptoms affecting all organ systems.17

  • Omalizumab (Xolair®)

Chemotherapy

In cases where the patient is diagnosed with an aggressive, systemic variant of mastocytosis, chemotherapy, more specifically D816V KIT Inhibitors, can be prescribed.18

FDA-Approved D816V KIT Inhibitors

  • Avapritinib (Ayvakit ®)19,20
  • Midostaurin (Rydapt ®)21,22

D816V KIT Inhibitors Currently in Clinical Trial

  • Cladribine (Leustatin®, Leustat®, Litak®)23
  • Dasatinib (Sprycel®)24
  • Imatinib (Gleevec®)25
  • INF - α 2b (Interferon Alpha 2b)26
  • Masitinib (Masivet®)27
  • Nilotinib (Tasigna®)28
  • Ripretinib (Qinlock®)29

PPIs (Proton Pump Inhibitors) to Treat GERD (Gastroesophageal Reflux Disease)

PPIs (proton pump inhibitors) can be used to treat GERD that develops as a result of mast cell disease.30

  • Exlansoprazol (Dexilant®)
  • Esomeprazole (Nexium®)
  • Lansoprazole (Prevacid®)
  • Omeprazole (Prilosec®)
  • Pantoprazole (Protonix®)
  • Rabeprazole (Aciphex®)

PUVA Phototherapy for the Treatment of Severe Skin Symptoms

PUVA combination therapy using oral psoralen plus ultraviolet-A light can be used in cases of severe skin symptoms to help treat skin lesions.31

Emergency Treatment

Emergency treatment for mast cell disease is administered in the case of anaphylactic shock. All patients should create an emergency treatment plan with their physician that includes:

  • Increasing H1 and H2 blockers
  • Taking Benadryl®
  • Using an inhaler
  • Using a self-injectable Epi-Pen®

Learn more about emergency care for mast cell disorders.

References:

  1. Maintz L and Novak N. Histamine and histamine intolerance. The American Journal of Clinical Nutrition. 2007:85(5);1185–1196. https://doi.org/10.1093/ajcn/85.5.1185
  2. Valent P, Akin C, Gleixner KV, et al. Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches. Int J Mol Sci. 2019;20(12):2976. https://doi:10.3390/ijms20122976
  3. Criado PR, Criado RF, Maruta CW, Machado Filho Cd. Histamine, histamine receptors and antihistamines: new concepts. An Bras Dermatol. 2010;85(2):195-210. https://pubmed.ncbi.nlm.nih.gov/20520935/
  4. Stefanini GF, Saggioro A, Alvisi V, Angelini G, Capurso L, di Lorenzo G, Dobrilla G, Dodero M, Galimberti M, Gasbarrini G, et al. Oral cromolyn sodium in comparison with elimination diet in the irritable bowel syndrome, diarrheic type. Multicenter study of 428 patients. Scand J Gastroenterol. 1995 Jun;30(6):535-41. https://pubmed.ncbi.nlm.nih.gov/7569760/
  5. Sokol KC, Amar NK, Starkey J, Grant JA. Ketotifen in the management of chronic urticaria: resurrection of an old drug. Ann Allergy Asthma Immunol. 2013;111(6):433-436. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4309375/
  6. Shaik Y, Caraffa A, Ronconi G, Lessiani G, Conti P. Impact of polyphenols on mast cells with special emphasis on the effect of quercetin and luteolin. Cent Eur J Immunol. 2018;43(4):476-481. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384425/
  7. Weng Z, Zhang B, Asadi S, Sismanopoulos N, Butcher A, Fu X, Katsarou-Katsari A, Antoniou C, Theoharides TC. Quercetin Is More Effective than Cromolyn in Blocking Human Mast Cell Cytokine Release and Inhibits Contact Dermatitis and Photosensitivity in Humans. PLoS One. 2012;7(3):e33805. https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0033805
  8. Minutello K, Gupta V. Cromolyn Sodium. [Updated 2022 Jan 8]. In: StatPearls (Internet). Treasure Island (FL): StatPearls Publishing; 2022 Jan-. https://www.ncbi.nlm.nih.gov/books/NBK557473/.
  9. Edwards AM, Capková S. Oral and topical sodium cromoglicate in the treatment of diffuse cutaneous mastocytosis in an infant. BMJ Case Rep. 2011;2011. https://pubmed.ncbi.nlm.nih.gov/7569760/
  10. Ang DC, Hilligoss J, Stump T. Mast Cell Stabilizer (Ketotifen) in Fibromyalgia: Phase 1 Randomized Controlled Clinical Trial. Clin J Pain. 2015;31(9):836-842. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4417653/#:~:text=Ketotifen%20is%20a%20mast%20cell,%2Dstabilizing%20properties%20(25)
  11. Shaik-Dasthagirisaheb, Yazdani. Relationship between Vitamin C, Mast Cells and Inflammation. Journal of Nutrition & Food Sciences. 06. https://www.researchgate.net/publication/293799498_Relationship_between_Vitamin_C_Mast_Cells_and_Inflammation
  12. Liu ZQ, Li XX, Qiu SQ, Yu Y, Li MG, Yang LT, Li LJ, Wang S, Zheng PY, Liu ZG, Yang PC. Vitamin D contributes to mast cell stabilization. Allergy. 2017;72(8):1184-1192. https://pubmed.ncbi.nlm.nih.gov/27998003/
  13. Cikler E, Ersoy Y, Cetinel S, Ercan F. The leukotriene d4 receptor antagonist, montelukast, inhibits mast cell degranulation in the dermis induced by water avoidance stress. Acta Histochem. 2009;111(2):112-8. https://pubmed.ncbi.nlm.nih.gov/18617226/
  14. Cefali EA, Simmons PD, Stanek EJ, McGovern ME, Kissling CJ. Aspirin reduces cutaneous flushing after administration of an optimized extended-release niacin formulation. Int J Clin Pharmacol Ther. 2007;45(2):78-88. https://pubmed.ncbi.nlm.nih.gov/17323787/#:~:text=Aspirin%20also%20significantly%20reduced%20intensity,flushing%20incidence%2C%20intensity%20and%20duration
  15. Butterfield JH, Weiler CR. Prevention of mast cell activation disorder-associated clinical sequelae of excessive prostaglandin D(2) production. Int Arch Allergy Immunol. 2008;147(4):338-43. https://pubmed.ncbi.nlm.nih.gov/18622141/
  16. Huang ES, Strate LL, Ho WW, Lee SS, Chan AT. Long-term use of aspirin and the risk of gastrointestinal bleeding. Am J Med. 2011;124(5):426-433. https://pubmed.ncbi.nlm.nih.gov/21531232/#:~:text=Conclusion%3A%20Regular%20aspirin%20use%20is,%2D%20and%20long%2Dterm%20users
  17. Weiss SL, Hyman JB, Carlson GS, Coop CA. Long-Term Successful Treatment of Indolent Systemic Mastocytosis With Omalizumab. Fed Pract. 2021;38(1):44-48. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7870274/
  18. Gilreath JA, Tchertanov L, Deininger MW. Novel approaches to treating advanced systemic mastocytosis. Clin Pharmacol. 2019 Jul 10;11:77-92. https://pubmed.ncbi.nlm.nih.gov/31372066/
  19. US Food and Drug Administration. FDA approves avapritinib for advanced systemic mastocytosis. Published June 16, 2021. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-avapritinib-advanced-systemic-mastocytosis
  20. DeAngelo DJ, Radia DH, George TI, Robinson WA, Quiery AT, Drummond MW, Bose P, Hexner EO, Winton EF, Horny HP, Tugnait M, Schmidt-Kittler O, Evans EK, Lin HM, Mar BG, Verstovsek S, Deininger MW, Gotlib J. Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial. Nat Med. 2021;27(12):2183-2191. https://pubmed.ncbi.nlm.nih.gov/34873347/
  21. Kasamon YL, Ko CW, Subramaniam S, Ma L, Yang Y, Nie L, Shord S, Przepiorka D, Farrell AT, McKee AE, Pazdur R. FDA Approval Summary: Midostaurin for the Treatment of Advanced Systemic Mastocytosis. Oncologist. 2018;23(12):1511-1519. https://pubmed.ncbi.nlm.nih.gov/30115735/#:~:text=In%20April%202017%2C%20the%20U.S.,mast%20cell%20leukemia%20(MCL)
  22. Valent P, Akin C, Hartmann K, George TI, Sotlar K, Peter B, Gleixner KV, Blatt K, Sperr WR, Manley PW, Hermine O, Kluin-Nelemans HC, Arock M, Horny HP, Reiter A, Gotlib J. Midostaurin: a magic bullet that blocks mast cell expansion and activation. Ann Oncol. 2017;28(10):2367-2376. https://pubmed.ncbi.nlm.nih.gov/28945834/#:~:text=Midostaurin%2C%20also%20known%20as%20PKC412,inhibits%20IgE%2Ddependent%20mediator%20secretion.&text=Keywords%3A%20IgE%3B%20mast%20cell%20activation,%3B%20mast%20cells%3B%20targeted%20drugs
  23. Akin C. Cladribine for mastocytosis: benefits and risks. Blood. 2015 Aug;126(8):931-2. https://pubmed.ncbi.nlm.nih.gov/26294717/
  24. Verstovsek S, Tefferi A, Cortes J, et al. Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis. Clin Cancer Res. 2008;14(12):3906-3915. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018899/
  25. Alvarez-Twose I, Matito A, Morgado JM, et al. Imatinib in systemic mastocytosis: a phase IV clinical trial in patients lacking exon 17 KIT mutations and review of the literature. Oncotarget. 2016;8(40):68950-68963. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5620310/
  26. Butterfield JH, Tefferi A, Kozuh GF. Successful treatment of systemic mastocytosis with high-dose interferon-alfa: long-term follow-up of a case. Leuk Res. 2005 Feb;29(2):131-4. https://pubmed.ncbi.nlm.nih.gov/15607359/
  27. Lortholary O, Chandesris MO, Bulai Livideanu C, et al. Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study. Lancet. 2017;389(10069):612-620. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985971/
  28. Blay JY, von Mehren M. Nilotinib: a novel, selective tyrosine kinase inhibitor [published correction appears in Semin Oncol. 2011 Jun;38(3):467]. Semin Oncol. 2011;38 Suppl 1(0 1):S3-S9. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4004101/#:~:text=Activity%20against%20KIT%20and%20mutant,cell%20lines%20(Table%201)
  29. Gupta A, Singh J, García-Valverde A, Serrano C, Flynn DL, Smith BD. Ripretinib and MEK Inhibitors Synergize to Induce Apoptosis in Preclinical Models of GIST and Systemic Mastocytosis. Molecular Cancer Therapeutics. 2021:20(7). https://mct.aacrjournals.org/content/20/7/1234
  30. Wauters L, Ceulemans M, Frings D, Lambaerts M, Accarie A, Toth J, Mols R, Augustijns P, De Hertogh G, Van Oudenhove L, Tack J, Vanuytsel T. Proton Pump Inhibitors Reduce Duodenal Eosinophilia, Mast Cells, and Permeability in Patients With Functional Dyspepsia. Gastroenterology. 2021 Apr;160(5):1521-1531.e9. https://pubmed.ncbi.nlm.nih.gov/33346007/
  31. Pyatilova P, Teplyuk N, Olisova O, Kovrigina A, Gadaev I, Kolkhir P. Efficacy of PUVA in Russian patients with mastocytosis: a case series and review of literature. Int J Dermatol. 2018 Sep;57(9):e59-e64. https://pubmed.ncbi.nlm.nih.gov/30133755/

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