Presentation of untreated systemic mastocytosis as recurrent, pulseless-electrical-activity cardiac arrests resistant to cardiac pacemaker

Joseph H. Butterfield, Catherine R. Weiler

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)130-134
Number of pages5
JournalInternational Archives of Allergy and Immunology
Volume163
Issue number2
DOIs
StatePublished - Feb 2014

Fingerprint

Systemic Mastocytosis
montelukast
Heart Arrest
Mast Cells
Cutaneous Mastocytosis
Cetirizine
Mastocytosis
Cell Degranulation
Tryptases
Histamine H1 Receptors
Cromolyn Sodium
Histamine H2 Receptors
Ranitidine
Hypotension
Epinephrine
Aspirin
Bone Marrow
Maintenance
Breast Neoplasms
Neoplasm Metastasis

Keywords

  • Pacemaker
  • Pulseless-electrical-activity arrests
  • Systemic mastocytosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Presentation of untreated systemic mastocytosis as recurrent, pulseless-electrical-activity cardiac arrests resistant to cardiac pacemaker. / Butterfield, Joseph H.; Weiler, Catherine R.

In: International Archives of Allergy and Immunology, Vol. 163, No. 2, 02.2014, p. 130-134.

Research output: Contribution to journalArticle

@article{b1dc480e71594d1eb892eeb4c58cba4e,
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.",
keywords = "Pacemaker, Pulseless-electrical-activity arrests, Systemic mastocytosis",
author = "Butterfield, {Joseph H.} and Weiler, {Catherine R.}",
year = "2014",
month = "2",
doi = "10.1159/000356487",
language = "English (US)",
volume = "163",
pages = "130--134",
journal = "International Archives of Allergy and Immunology",
issn = "1018-2438",
publisher = "S. Karger AG",
number = "2",

}

TY - JOUR

T1 - Presentation of untreated systemic mastocytosis as recurrent, pulseless-electrical-activity cardiac arrests resistant to cardiac pacemaker

AU - Butterfield, Joseph H.

AU - Weiler, Catherine R.

PY - 2014/2

Y1 - 2014/2

N2 - 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.

AB - 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.

KW - Pacemaker

KW - Pulseless-electrical-activity arrests

KW - Systemic mastocytosis

UR - http://www.scopus.com/inward/record.url?scp=84896865954&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84896865954&partnerID=8YFLogxK

U2 - 10.1159/000356487

DO - 10.1159/000356487

M3 - Article

C2 - 24335343

AN - SCOPUS:84896865954

VL - 163

SP - 130

EP - 134

JO - International Archives of Allergy and Immunology

JF - International Archives of Allergy and Immunology

SN - 1018-2438

IS - 2

ER -