Systemic Mastocytosis Medication

Updated: May 09, 2017
  • Author: Koyamangalath Krishnan, MD, FRCP, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Medication

Medication Summary

Tyrosine kinase inhibitors (eg, midostaurin, imatinib) have been approved by the FDA for various forms of mastocytosis. [10, 11]

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Antineoplastics, Tyrosine Kinase Inhibitor

Class Summary

The tyrosine kinase inhibitors midostaurin and imatinib have each been approved by the FDA for various types of mastocytosis. [10]

Midostaurin (Rydapt)

Midostaurin has demonstrated the ability to inhibit KIT signaling, cell proliferation, and histamine release and induce apoptosis in mast cells. It is indicated for adults with advanced mastocytosis which includes aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL).

Imatinib (Gleevec)

Imatinib is a protein-tyrosine kinase inhibitor that inhibits the BCR-ABL tyrosine kinase. It is also an inhibitor of the receptor tyrosine kinases for platelet-derived growth factor (PDGF) and stem cell factor (SCF), c-kit, and inhibits PDGF-and SCF-mediated cellular events. In vitro, imatinib inhibits proliferation and induces apoptosis in GIST cells, which express an activating c-kit mutation. It is indicated for adults with aggressive systemic mastocytosis without the D816V c-Kit mutation as determined with an FDA-approved test.

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Sympathomimetics

Class Summary

Sympathomimetic agents are used in the treatment of anaphylaxis.

Epinephrine (Adrenalin, Bronitin, EpiPen)

Drug of choice for treating anaphylactoid reactions. Has alpha-agonist effects that include increased peripheral vascular resistance, reversed peripheral vasodilatation, systemic hypotension, and vascular permeability. Beta-agonist effects of epinephrine include bronchodilatation, chronotropic cardiac activity, and positive inotropic effects.

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Mast Cell Stabilizers

Class Summary

Mast cell stabilizers prevent release of mediators from mast cells, which cause airway inflammation and bronchospasm.

Cromolyn sodium (Intal, NasalCrom)

Inhibits degranulation of sensitized mast cells following their exposure to specific antigens.

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Corticosteroids

Class Summary

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify body's immune response to diverse stimuli.

Prednisone (Deltasone, Orasone, Meticorten)

Immunosuppressant for the treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear neutrophil activity.

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