Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: a case report.

Publisher: BioMed Central Country of Publication: England NLM ID: 101293382 Publication Model: Electronic Cited Medium: Internet ISSN: 1752-1947 (Electronic) Linking ISSN: 17521947 NLM ISO Abbreviation: J Med Case Rep Subsets: MEDLINE

Original Publication: [London] : BioMed Central, [2007-

Diphenhydramine/*administration & dosage ; Diphenhydramine/*therapeutic use ; Histamine H1 Antagonists/*administration & dosage ; Histamine H1 Antagonists/*therapeutic use ; Imatinib Mesylate/*therapeutic use ; Mastocytosis/*drug therapy ; Protein Kinase Inhibitors/*therapeutic use; Adolescent ; Female ; Humans ; Imatinib Mesylate/administration & dosage ; Infusions, Parenteral ; Mastocytosis/physiopathology ; Protein Kinase Inhibitors/administration & dosage ; Quality of Life ; Treatment Outcome

Background: 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. 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 Presentation: An 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.
Conclusions: 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 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.

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Keywords: Continuous diphenhydramine infusion; Imatinib; Mast cell activation disease; Mast cell activation syndrome; Mastocytosis; Paucity of controlled trials

0 (Histamine H1 Antagonists)
0 (Protein Kinase Inhibitors)
8A1O1M485B (Imatinib Mesylate)
8GTS82S83M (Diphenhydramine)

Date Created: 20170426 Date Completed: 20180221 Latest Revision: 20181113

20231215

PMC5402659

10.1186/s13256-017-1278-3

28438191