CLINICAL ADVANCES IN MASTOCYTOSIS

Mastocytosis is a disease characterized by an abnormal proliferation o f tissue mast cells. The events primarily responsible for mast cell pr oliferation in mastocytosis are largely unknown, but a derangement of the network involving c-kit receptor and its natural ligand (stem cell factor, which promotes mast cell growth and differentiation in man) i s likely to have a primary role in this disease. Mastocytosis comprise s a wide spectrum of clinical conditions determined by the degree of m ast cell proliferation, the organ systems involved, the age at onset a nd the association with hematologic diseases. Mastocytosis can occur i n a pediatric or an adult form. In both groups of patients, the diseas e may be limited to the skin (cutaneous mastocytosis) or be systemic, involving predominantly the bone marrow and the gastrointestinal tract . The symptoms in patients with mastocytosis are generally related to the increased release of mast-cell-derived mediators, such as histamin e, prostaglandin D-2, peptide leukotrienes, platelet-activating factor , heparin and proteolytic enzymes. The measurement of these chemical m ediators (histamine, tryptase and prostaglandin D-2 and their metaboli tes) in body fluids is useful for the diagnosis and the laboratory eva luation of patients with systemic mastocytosis. As little is known abo ut the pathogenesis of the different forms of mastocytosis, the treatm ent of the majority of these patients is largely symptomatic.