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Mast Cell Clonal Disorders: Classification, Diagnosis and Management

View Article: PubMed Central - PubMed

ABSTRACT

Mast cell clonal disorders are characterized by the clonal proliferation of pathological mast cells as a result of somatic mutations in the KIT gene, most commonly the D816V mutation. Accumulation and degranulation of these cells causes a wide variety of symptoms. Mast cell clonal disorders can be divided into mastocytosis and monoclonal mast cell activation syndrome, depending of the level of clonality. The severity of mastocytosis varies from an indolent variant with a good prognosis, to an aggressive condition with short life expectancy. Diagnosis is based on demonstration of clonality and accumulation in the skin and in extracutaneous tissues. Treatment is highly individualized, and is based on the severity of the condition. Treatment of patients with indolent systemic mastocytosis is aimed at reducing symptoms, using histamine H1 and H2 receptor antagonists as a starting point. In addition, associated conditions such as osteoporosis must be treated. Treatment of advanced systemic mastocytosis is aimed at reducing mast cell load through cytoreductive therapy. The choice of such therapy depends on the KIT mutational status. Though currently there is no curative treatment available, promising new therapies such as midostaurin are emerging that have demonstrated success in reducing symptoms and improving quality of life.

No MeSH data available.


Related in: MedlinePlus

Add-on steps for mastocytosis treatment. Start treatment with H1 antagonists. Add H2 antagonists and/or cromoglicic acid in the case of GI symptoms or inadequately resolved pruritus. Leukotriene antagonists are added in the case of musculoskeletal pain or inadequately resolved pruritus. Tyrosine kinase inhibitors are thus far used only in patients with advanced SM.
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Fig1: Add-on steps for mastocytosis treatment. Start treatment with H1 antagonists. Add H2 antagonists and/or cromoglicic acid in the case of GI symptoms or inadequately resolved pruritus. Leukotriene antagonists are added in the case of musculoskeletal pain or inadequately resolved pruritus. Tyrosine kinase inhibitors are thus far used only in patients with advanced SM.

Mentions: Therapeutic options can be subdivided into antihistamines, leukotriene antagonists, cromoglicic acid systemic glucocorticosteroids, tyrosine kinase inhibitors (TKIs) and cytoreductive treatment. A combination of drugs is often necessary to achieve symptom control. An overview of the add-on steps in therapeutic options can be seen in Fig. 1.Fig. 1


Mast Cell Clonal Disorders: Classification, Diagnosis and Management
Add-on steps for mastocytosis treatment. Start treatment with H1 antagonists. Add H2 antagonists and/or cromoglicic acid in the case of GI symptoms or inadequately resolved pruritus. Leukotriene antagonists are added in the case of musculoskeletal pain or inadequately resolved pruritus. Tyrosine kinase inhibitors are thus far used only in patients with advanced SM.
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC5121168&req=5

Fig1: Add-on steps for mastocytosis treatment. Start treatment with H1 antagonists. Add H2 antagonists and/or cromoglicic acid in the case of GI symptoms or inadequately resolved pruritus. Leukotriene antagonists are added in the case of musculoskeletal pain or inadequately resolved pruritus. Tyrosine kinase inhibitors are thus far used only in patients with advanced SM.
Mentions: Therapeutic options can be subdivided into antihistamines, leukotriene antagonists, cromoglicic acid systemic glucocorticosteroids, tyrosine kinase inhibitors (TKIs) and cytoreductive treatment. A combination of drugs is often necessary to achieve symptom control. An overview of the add-on steps in therapeutic options can be seen in Fig. 1.Fig. 1

View Article: PubMed Central - PubMed

ABSTRACT

Mast cell clonal disorders are characterized by the clonal proliferation of pathological mast cells as a result of somatic mutations in the KIT gene, most commonly the D816V mutation. Accumulation and degranulation of these cells causes a wide variety of symptoms. Mast cell clonal disorders can be divided into mastocytosis and monoclonal mast cell activation syndrome, depending of the level of clonality. The severity of mastocytosis varies from an indolent variant with a good prognosis, to an aggressive condition with short life expectancy. Diagnosis is based on demonstration of clonality and accumulation in the skin and in extracutaneous tissues. Treatment is highly individualized, and is based on the severity of the condition. Treatment of patients with indolent systemic mastocytosis is aimed at reducing symptoms, using histamine H1 and H2 receptor antagonists as a starting point. In addition, associated conditions such as osteoporosis must be treated. Treatment of advanced systemic mastocytosis is aimed at reducing mast cell load through cytoreductive therapy. The choice of such therapy depends on the KIT mutational status. Though currently there is no curative treatment available, promising new therapies such as midostaurin are emerging that have demonstrated success in reducing symptoms and improving quality of life.

No MeSH data available.


Related in: MedlinePlus