montelukast and Mastocytosis--Systemic

The approach to clinical conundrums by an expert clinician is revealed through the presentation of an actual patient's case in an approach typical of a morning report. Similarly to patient care, sequential pieces of information are provided to the clinician, who is unfamiliar with the case. The focus is on the thought processes of both the clinical team caring for the patient and the discussant.

Topics: Acetates; Aged; Anti-Inflammatory Agents; Cyclopropanes; Diagnosis, Differential; Humans; Hypotension; Leukotriene Antagonists; Male; Mastocytosis, Systemic; Prednisone; Quinolines; Sulfides; Tachycardia

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.

Topics: Acetates; Aged, 80 and over; Aspirin; Cromolyn Sodium; Cyclopropanes; Death, Sudden, Cardiac; Drug Therapy, Combination; Electrocardiography; Female; Heart Rate; Histamine; Histamine H1 Antagonists; Histamine H2 Antagonists; Humans; Interleukin-2 Receptor alpha Subunit; Mast Cells; Mastocytosis, Systemic; Methylhistamines; Mutation; Pacemaker, Artificial; Quinolines; Recurrence; Stem Cell Factor; Sulfides; Tryptases

Topics: Acetates; Adult; Anti-Inflammatory Agents; Cromolyn Sodium; Cyclopropanes; Diarrhea; Drug Therapy, Combination; Enzyme Inhibitors; Exanthema; Female; Flushing; Histamine H2 Antagonists; Humans; Leukotriene Antagonists; Mastocytosis, Systemic; Prednisone; Protein-Tyrosine Kinases; Quinolines; Ranitidine; Staurosporine; Sulfides; Tryptases; Vomiting

Topics: Abdominal Pain; Acetates; Anti-Asthmatic Agents; Anti-Inflammatory Agents; Asthma; Budesonide; Capillary Permeability; Cough; Cyclopropanes; Diarrhea; Dyspnea; Female; Humans; Lung; Mast Cells; Mastocytosis, Systemic; Middle Aged; Nedocromil; Quinolines; Sulfides

Topics: Acetates; Anti-Inflammatory Agents; Cyclopropanes; Drug Therapy, Combination; Humans; Infant; Leukotriene Antagonists; Male; Mastocytosis, Systemic; Prednisone; Quinolines; Sulfides