title: Presentation of untreated systemic mastocytosis as recurrent, pulseless-electrical-activity cardiac arrests resistant to cardiac pacemaker.
abstract: Recurrent, pulseless-electrical-activity (PEA) cardiac arrests were the novel presentation of untreated systemic mastocytosis in an 85-year-old woman who lacked cutaneous findings of mastocytosis. Despite prior implantation of a dual-chamber cardiac pacemaker 3 weeks previously for similar spells, she experienced a PEA arrest accompanied by flushing, increased urinary N-methylhistamine excretion and serum tryptase values on the day of presentation to our clinic. Bone marrow biopsy findings conducted to rule out breast cancer metastases showed 30% mast cell infiltration, aberrant expression of CD25 and a positive c-kit Asp816Val mutation. Treatment with a combination of H1 and H2 receptor blockers reduced flushing and eliminated hypotension. Maintenance medication included aspirin, cetirizine, ranitidine, montelukast, oral cromolyn sodium and an epinephrine autoinjector (as needed). At 6-month follow-up, the patient remained free of PEA arrests, flushing, or any clinical signs of mastocytosis or mast cell degranulation. PEA cardiac arrests may therefore be a presenting sign of untreated systemic mastocytosis.
authors: Division of Allergic Diseases and the Mayo Clinic Program for Mast Cell and Eosinophil Disorders, Mayo Clinic, Rochester, Minn., USA.
authors: Joseph H
authors: JH
authors: Butterfield
authors: Catherine R
authors: CR
authors: Weiler
mesh_terms: D000085:Acetates / Q000008:administration & dosage; D000369:Aged, 80 and over; D001241:Aspirin / Q000008:administration & dosage; D004205:Cromolyn Sodium / Q000008:administration & dosage; D003521:Cyclopropanes; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000517:prevention & control; D004359:Drug Therapy, Combination / Q000379:methods*; D004562:Electrocardiography; D005260:Female; D006339:Heart Rate; D006632:Histamine / Q000378:metabolism*; D006634:Histamine H1 Antagonists / Q000008:administration & dosage; D006635:Histamine H2 Antagonists / Q000008:administration & dosage; D006801:Humans; D053645:Interleukin-2 Receptor alpha Subunit / Q000378:metabolism; D008407:Mast Cells / Q000473:pathology*; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D008761:Methylhistamines / Q000378:metabolism; D009154:Mutation / Q000235:genetics; D010138:Pacemaker, Artificial / Q000706:statistics & numerical data; D011804:Quinolines / Q000008:administration & dosage; D012008:Recurrence; D019089:Stem Cell Factor / Q000235:genetics; D013440:Sulfides; D053802:Tryptases / Q000097:blood
chemical_list: D000085:Acetates; D003521:Cyclopropanes; D006634:Histamine H1 Antagonists; D006635:Histamine H2 Antagonists; D053645:Interleukin-2 Receptor alpha Subunit; D008761:Methylhistamines; D011804:Quinolines; D019089:Stem Cell Factor; D013440:Sulfides; D006632:Histamine; D053802:Tryptases; C093875:montelukast; D004205:Cromolyn Sodium; D001241:Aspirin; C048140:N-methylhistamine