Description

Systemic mastocytosis (SM) is a heterogeneous group of rare hematologic neoplasms that are characterized by clonal proliferation of mast cells in the bone marrow and extracutaneous organs, which can cause symptoms due to mast-cell mediator release and end-organ damage due to mast-cell infiltration. Mastocytosis is classified as a distinct entity by the WHO because of its unique clinicopathological features. Over 95% of patients harbor the somatic KIT driver mutation D816V. This book focuses on the diagnosis of SM, with up-to-date practical guidance on the management of patients with SM, who may present to a variety of healthcare providers on their diagnostic journey. It will be invaluable to clinicians, medical students, nurses, pharmacists and scientists in understanding the clinical, diagnostic and management challenges associated with SM. Table of Contents: -Definitions and classification -Epidemiology and pathophysiology -Diagnosis -Management of non-advanced systemic mastocytosis -Management of advanced systemic mastocytosis -Future directions