Management of adult mastocytosis

Abstract

Introduction: Mastocytosis comprises a heterogeneous group of disorders characterized by the presence of abnormal mast cells (MCs) in one or more tissues. The great heterogeneity of mastocytosis is determined by the clinical and biological diversity observed in some cases as reflected, for example, by the existence of cases arising in childhood versus those who have an adult-onset disease, patients with mastocytosis restricted to skin versus those with systemic involvement or cases with a chronic, indolent course versus those who suffer from aggressive or leukemic manifestations. This broad spectrum of the disease translates into different therapeutic options, which should be carefully selected in every case based on several factors, such as the patient's age, the frequency, type and severity of MC mediator release symptoms, the overall MC burden, the presence of poor prognosis markers or the detection of specific molecular targets.

Areas covered: This comprehensive review is focused on the different therapeutic strategies used in adult mastocytosis, including antimediator drugs, cytoreductive treatments and tyrosine kinase inhibitors.

Expert opinion: Herein, this review highlights several evidence-based aspects related to the management of adult mastocytosis, with special emphasis on the recommendations from the Spanish Network on Mastocytosis and the importance of using highly sensitive molecular diagnostic methods to improve the selection of patient candidates to targeted therapies such as imatinib.

Keywords::

• antimediator drugs
• c-kit
• cladribine
• imatinib
• interferon
• mast cell
• mastocytosis
• midostaurin
• therapy
• tyrosine kinase

Notes

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