Initial Treatment for Mastocytosis

The primary initial treatment for mastocytosis is H1 antihistamines to control symptoms such as pruritus, flushing, urticaria, and tachycardia. 1

First-Line Treatment Approach

H1 antihistamines are recommended by the American College of Dermatology as the first-line treatment to manage common symptoms including pruritus, flushing, urticaria, and tachycardia 1
Combined H1 and H2 antihistamine therapy is effective for controlling severe pruritus, wheal formation, and gastric hypersecretion in patients with more pronounced symptoms 1, 2
Avoidance of known triggering factors is essential as a non-pharmacological component of initial management to prevent mast cell activation episodes 1

If H2 antihistamines are insufficient for controlling gastrointestinal symptoms, proton pump inhibitors should be added 1
Cromolyn sodium oral solution (200 mg four times daily) has shown clinically significant improvement in gastrointestinal symptoms (diarrhea, abdominal pain) in randomized controlled trials 3
Clinical improvement with cromolyn sodium typically occurs within 2-6 weeks of treatment initiation 3

Water-soluble sodium cromolyn cream or aqueous-based sodium cromolyn skin lotion can decrease pruritus and flaring of skin lesions 1
Topical treatments should be used as adjuncts to systemic antihistamine therapy for cutaneous mastocytosis 4

For acute mast cell activation attacks involving hypotension, wheezing, or laryngeal edema, epinephrine should be administered intramuscularly in a recumbent position 1, 4
Patients with history of severe reactions should be prescribed self-injectable epinephrine and educated on its use 2
Prolonged activation episodes may require short courses of corticosteroids 4

Pre-anesthetic treatment with anxiolytic agents, antihistamines, and possibly corticosteroids is recommended to reduce the frequency/severity of mast cell activation events during surgery 1
Safer perioperative drugs include propofol, sevoflurane, isoflurane, fentanyl, remifentanil, lidocaine, and bupivacaine 1

Cytoreductive therapy should be avoided in initial treatment except in life-threatening aggressive variants of mastocytosis 1, 5
For severe cases of systemic mastocytosis during pregnancy refractory to conventional therapy, interferon-alfa can be considered 1

High doses of H1 antihistamines may cause cardiotoxicity, requiring careful dosing and monitoring 1
The heterogeneity of mastocytosis requires recognition of specific disease patterns to guide appropriate therapy 2
Combination therapy with H1 and H2 receptor antagonists (such as ketotifen and ranitidine) has been shown to inhibit mediator release from mast cells effectively 6
Treatment should be escalated in a stepwise fashion, starting with antihistamines and adding additional agents only if symptoms persist 4