Table 5

Treatment options for mast cell activation disease.

Basic therapy (continuous oral combination therapy to reduce mast cell activity)

• H1-histamine receptor antagonist (to block activating H1-histamine receptors on mast cells; to antagonize H1-histamine receptor-mediated symptoms)

• H2- histamine receptor antagonist (to block activating H2-histamine receptors on mast cells; to antagonize H2-histamine receptor-mediated symptoms)

• Cromolyn sodium (stabilising mast cells)

• Slow-release Vitamin C (increased degradation of histamine; inhibition of mast cell degranulation; not more than 750 mg/day)

• If necessary, ketotifen to stabilise mast cells and to block activating H1-histamine receptors on mast cells


Symptomatic treatment options (orally as needed)

Headache⇒ paracetamol; metamizole; flupirtine

Diarrhea⇒ colestyramine; nystatin; montelukast; 5-HT3 receptor inhibitors (eg. ondansetron); incremental doses (50-350 mg/day; extreme caution because of the possibility to induce mast cell degranulation) of acetylsalicylic acid; (in steps test each drug for 5 days until improvement of diarrhea)

Colicky abdominal paindue to distinct meteorism ⇒ metamizole; butylscopolamine

Nausea⇒ metoclopramide; dimenhydrinate; 5-HT3 receptor inhibitors; icatibant

Respiratory symptoms(mainly increased production of viscous mucus and obstruction with compulsive throat clearing) ⇒ montelukast; urgent: short-acting ß-sympathomimetic

Gastric complaints⇒ proton pump inhibitors (de-escalating dose finding)

Osteoporosis, osteolysis, bone pain⇒ biphosphonates ([51]; vitamin D plus calcium application is second-line treatment in MCAD patients because of limited reported success and an increased risk for developing kidney and ureter stones; [52])

Non-cardiac chest pain⇒ when needed, additional dose of a H2-histamine receptor antagonist; also, proton pump inhibitors for proven gastroesophageal reflux

Tachycardia⇒ verapamil; AT1-receptor antagonists; ivabradin

Neuropathic pain and paresthesia⇒ α-lipoic acid

Interstitial cystitis⇒ pentosan, amphetamines

Sleep-onset insomnia/sleep-maintenance insomnia⇒ triazolam/oxazepam

Conjunctivitis⇒ exclusion of a secondary disease; otherwise preservative-free eye drops with glucocorticoids for brief courses

Hypercholesterolemia⇒ (does not depend on the composition of the diet) therapeutic trial with HMG-CoA reductase inhibitors (frequently ineffective)

Elevated prostaglandin levels, persistant flushing⇒ incremental doses of acetylsalicylic acid (50-350 mg/day; extreme caution because of the possibility to induce mast cell degranulation)


All drugs should be tested for tolerance in a low single dose before therapeutic use, if their tolerance in the patient is not known from an earlier application.

Molderings et al. Journal of Hematology & Oncology 2011 4:10   doi:10.1186/1756-8722-4-10

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