Abstract

BackgroundWe present the first full case report of the treatment of mast cell activation syndrome with continuous diphenhydramine infusion, which resulted in the improvement of anaphylactic reactions and a decrease in hospital readmission. Furthermore, the patient received imatinib in the absence of the KIT-D816V mutation, which led to further improvement of quality of life. Currently, we are trying to wean this patient off diphenhydramine; if successful, this attempt will represent the first reported case.Case presentationAn 18-year-old white girl presented with a flare of mast cell activation syndrome and received epinephrine and steroids. She had failed multiple previous therapies, and her quality of life was affected due to two to three flares/week. She was started on continuous diphenhydramine infusion and imatinib, which led to a decrease in hospital admissions and marked improvement in her quality of life.ConclusionsContinuous diphenhydramine infusion can provide promising outcomes following the failure of intermittent antihistamine dosing in patients with severe mast cell activation syndrome. Initiating continuous diphenhydramine infusion may be helpful in an intensive care setting when the patient is particularly prone to anaphylaxis and/or the resources needed to manage anaphylaxis are not available outside the intensive care unit. Furthermore, imatinib provides benefits in KIT-D816V-negative mast cell disorders due to other unknown mutations.

Highlights

  • We present the first full case report of the treatment of mast cell activation syndrome with continuous diphenhydramine infusion, which resulted in the improvement of anaphylactic reactions and a decrease in hospital readmission

  • Continuous diphenhydramine infusion can provide promising outcomes following the failure of intermittent antihistamine dosing in patients with severe mast cell activation syndrome

  • Initiating continuous diphenhydramine infusion may be helpful in an intensive care setting when the patient is prone to anaphylaxis and/or the resources needed to manage anaphylaxis are not available outside the intensive care unit

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Summary

Conclusions

No randomized controlled trials have been performed using patients with this disease, and the therapeutic data are all anecdotal. All such cases should be reported to increase awareness about possible therapeutics. Author details 1Department of Internal Medicine, Abington Jefferson Health, 1200 Old York Road, Abington, PA 19001, USA. Molderings GJ, Haenisch B, Brettner S, Homann J, Menzen M, Dumoulin FL, et al Pharmacological treatment options for mast cell activation disease. H1-antihistamines for primary mast cell activation syndromes: a systematic review. Corticosteroid treatment reduces mast cell numbers in inflammatory bowel disease. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. Omalizumab treatment of systemic mast cell activation disease: experiences from four cases.

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