Contemporary challenges in mastocytosis

H. David Pettigrew, Suzanne S Teuber, James S. Kong, M. Eric Gershwin

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Mastocytosis denotes a wide range of disorders characterized by having abnormal growth and accumulation of mast cells. Mast cells contain histamine and other inflammatory mediators, which have diverse actions within the body, and play crucial roles in acquired and innate immunity. The diverse actions of these inflammatory mediators can lead to puzzling symptoms in individuals with mastocytosis. These symptoms can include flushing, pruritis, nausea, vomiting, abdominal pain, diarrhea, vascular instability, and headache. These clinical features generally divide into cutaneous and systemic manifestations, giving rise to the two divisions of mastocytosis: cutaneous mastocytosis (CM) and systemic mastocytosis. CM has a highly favorable clinical prognosis. Systemic mastocytosis has a range of severity, with the milder forms often remaining chronic conditions, while the severe forms have rapid complex courses with poor prognoses. Generally, treatment is aimed at avoiding mast cell degranulation, inhibiting the actions of the constitutive mediators released by mast cells and, in severe cases, cytoreductive and polychemotherapeutic agents. Behavioral intervention includes avoidance of triggers, such as heat, cold, pressure, exercise, sunlight, and strong emotions. Treatment for released histamine and other inflammatory mediators includes H1 antihistamines, H2 antihistamines, proton pump inhibitors, anti-leukotriene agents, and injectible epinephrine (for possible anaphylaxis). For severe cases, treatment includes cytoreductive agents (interferon alpha, glucocorticoids, and cladribine) and polychemotherapeutic agents (daunomycin, etoposide, and 6-mercaptopurine). For very specific and severe cases, tyrosine kinase inhibitors, imatinib and midostaurine, have shown promise.

Original languageEnglish (US)
Pages (from-to)125-134
Number of pages10
JournalClinical Reviews in Allergy and Immunology
Volume38
Issue number2-3
DOIs
StatePublished - Apr 2010

Fingerprint

Mastocytosis
Mast Cells
Cutaneous Mastocytosis
Systemic Mastocytosis
Histamine Antagonists
Histamine
Vascular Headaches
Cladribine
Skin Manifestations
Cell Degranulation
6-Mercaptopurine
Daunorubicin
Leukotrienes
Proton Pump Inhibitors
Sunlight
Adaptive Immunity
Anaphylaxis
Etoposide
Pruritus
Innate Immunity

Keywords

  • IgE
  • Mastocytosis
  • Tryptase

ASJC Scopus subject areas

  • Immunology and Allergy

Cite this

Contemporary challenges in mastocytosis. / Pettigrew, H. David; Teuber, Suzanne S; Kong, James S.; Gershwin, M. Eric.

In: Clinical Reviews in Allergy and Immunology, Vol. 38, No. 2-3, 04.2010, p. 125-134.

Research output: Contribution to journalArticle

Pettigrew, H. David ; Teuber, Suzanne S ; Kong, James S. ; Gershwin, M. Eric. / Contemporary challenges in mastocytosis. In: Clinical Reviews in Allergy and Immunology. 2010 ; Vol. 38, No. 2-3. pp. 125-134.
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