4.1 Article

New Insights into Clonal Mast Cell Disorders Including Mastocytosis

Journal

Publisher

W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.iac.2018.04.014

Keywords

Mastocytosis; Mast cell; Clonal mast cell disorders; Monoclonal mast cell activation syndrome; Mast cell sarcoma; Tryptase; Mast cell leukemia

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Mastocytosis is a heterogeneous group of neoplasms that involve the clonal expansion of mast cells into one or more organ systems, which typically involves the skin and hematopoietic systems. Systemic mastocytosis consists of a multifocal infiltration of mast cells into various noncutaneous tissue sites, especially the bone marrow. Diagnosis requires tissue confirmation, and algorithms have been developed to assist clinicians in this process. The current classification system focuses on delineating prognostic categories. Therapeutic approaches include symptomatic management, prevention of complications, and, in advanced disease, cytoreductive therapy.

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