Intervention with epinephrine in hypotension associated with mastocytosis

doi:10.1016/0091-6749(83)90098-2

Journal of Allergy and Clinical Immunology

Volume 71, Issue 2, February 1983, Pages 189-192

https://doi.org/10.1016/0091-6749(83)90098-2 Get rights and content

Abstract

The occurrence of the episodes of vasodilatory hypotension can be a life-threatening manifestation of systemic mastocytosis. This article describes the reversal by epinephrine of episodes of severe hypotension in two hospitalized patients with mastocytosis. Recognition of the efficacy of epinephrine in hypotension associated with mastocytosis can be important when other methods fail to restore hemodynamic stability.

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Cited by (76)

Nontryptase Urinary and Hematologic Biomarkers of Mast Cell Expansion and Mast Cell Activation: Status 2022
2022, Journal of Allergy and Clinical Immunology: In Practice
Citation Excerpt :
Aspirin doses in several of these studies were relatively high, up to 4 g/d.28 A later survey of aspirin use in 20 patients with SM confirmed that much lower doses of aspirin (generally 81-325 mg twice a day) were sufficient to reduce elevated 24-hour excretion levels of urinary 11β-PGF2α to normal in most patients with SM.44 Although no fatalities from aspirin ingestion have been reported in SM or MCAS, it was recognized in earlier and subsequent reports that aspirin was capable of triggering MC degranulation often with severe associated symptoms.13,45-47 This side effect occurs in 5% to 10% or less of patients with SM.44
Quantitation of urinary metabolites of histamine, prostaglandin D₂, and leukotriene E₄ can fill the gap in our current efforts to improve diagnosis and management of symptomatic patients with systemic mastocytosis, and/or mast cell activation syndrome, In addition, patients symptomatic due to mast cell activation but who do not meet all the criteria for mast cell activation syndrome can have elevated baseline mediator metabolites. Serum tryptase levels have been the workhorse in diagnosing these disorders, but it has several drawbacks including the need to obtain acute and baseline samples, which require 2 visits to health care facilities and 2 venipunctures. Recently, increased baseline tryptase level has been reported in hereditary alpha tryptasemia, complicating diagnostic possibilities of an increased baseline tryptase level. Furthermore, no treatment can specifically be targeted at tryptase itself. In contrast, the finding of 1 or more elevated urinary levels of histamine, prostaglandin D₂, and/or leukotriene E₄ metabolites (1) greatly narrows diagnostic possibilities for causes of symptoms; (2) informs the practitioner what specific metabolic pathways are involved; and (3) targets the treatment in a specific, direct fashion. As a bonus, baseline spot/random urine samples can be obtained by the patients themselves and repeated at exactly the correct time when symptoms occur.
Anaphylaxis and Mast Cell Disorders
2022, Immunology and Allergy Clinics of North America
Citation Excerpt :
Acute episodes of SA in patients with MC disorders should be treated in the same manner as other forms of anaphylaxis.20,68,69 Roberts and colleagues70,71 stressed the unique role of epinephrine in the treatment of SA in a patient with SM who was refractory to vasopressor therapy with dopamine, yet quickly improved with epinephrine. Intramuscular (IM) epinephrine is the drug of choice.
Neuroendocrine Gastrointestinal and Lung Tumors (Carcinoid Tumors), the Carcinoid Syndrome, and Related Disorders
2015, Williams Textbook of Endocrinology
Mastocytosis
2014, Immunology and Allergy Clinics of North America
Citation Excerpt :
This situation is likely caused by the inability of these agents to completely block histamine and other mast cell mediators. Prompt administration of epinephrine is indicated for treatment of hypotensive episodes, which may be spontaneous or observed after insect stings and injection of contrast media.50 Patients should be trained in self-administration of injectable epinephrine.
Expanding spectrum of mast cell activation disorders: Monoclonal and idiopathic mast cell activation syndromes
2013, Clinical Therapeutics
In recent years, 2 new syndromes of mast cell activation have been described in patients with episodes of mast cell mediator release that range from flushing and abdominal cramping to anaphylaxis: monoclonal mast cell activation syndrome (MMAS) and idiopathic mast cell activation syndrome (MCAS).
This review will discuss these 2 new syndromes in the larger context of mast cell activation disorders as well as the diagnostic and treatment approaches for these conditions.
PubMed was searched using the following terms: mast cell activation disorder, mast cell activation syndrome, and clonal mast cell. Only English-language articles published up until February 27, 2013, were considered.
MMAS has been diagnosed in patients with systemic reactions to hymenoptera stings and elevated baseline serum tryptase as well as in patients with unexplained episodes of anaphylaxis. A bone marrow biopsy establishes the diagnosis by revealing the presence of monoclonal mast cells that carry the D816V KIT mutation and/or express CD25 while the diagnostic requirements for systemic mastocytosis are not met. MCAS affects predominantly women in whom no mast cell abnormality or external triggers account for their episodes of mast cell activation. MCAS is a diagnosis of exclusion, and primary and secondary mast cell activation disorders as well as idiopathic anaphylaxis have to be ruled out before making the diagnosis. Patients with MCAS and MMAS are treated in a stepwise fashion with drugs that block the effects of mediators released by mast cells on activation. One third of MCAS patients experience complete resolution of symptoms with treatment, while one third have a major response and one third a minor response to treatment. A combination of drugs is usually necessary to achieve symptom control. No drug trial has been performed in patients with MMAS and MCAS.
MMAS and MCAS are 2 newly described, rare syndromes of mast cell activation. Further studies will be necessary to better understand the cause of these conditions and their natural evolution and to validate and improve the treatment approach. Research should also focus on developing drugs with the potential to cure these debilitating disorders. To achieve these goals, centers with expertise in mast cell activation disorders are essential as they allow for a critical mass of these patients to be enrolled in studies while providing those patients with the most up-to-date diagnostic procedures and treatment strategies.
Skin and Bone Disorders
2012, Anesthesia and Uncommon Diseases: Sixth Edition

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^☆: This work was supported by grants GM15431 and MO1R R0095 from the National Institutes of Health.

^∗: Dr. Oates is the Joe and Morris Werthan Professor of Investigative Medicine.

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Original articleIntervention with epinephrine in hypotension associated with mastocytosis☆

Abstract

Am J Med

Am J Med

Am J Med

Clin Chim Acta

Methods Enzymol

Prostaglandins

J Biol Chem

Prostaglandins

Prostaglandins

Histamine and mastocytosis

The mastocytosis syndrome. Clinical and biological studies

Ann Intern Med

Fatal anaphylaxis in systemic mastocytosis

J Clin Phathol

Increased production of protaglandin D2 in patients with systemic mastocytosis

N Engl J Med

Original article
Intervention with epinephrine in hypotension associated with mastocytosis☆

Increased production of protaglandin D₂ in patients with systemic mastocytosis