Treatment of systemic past cell disorders

Despite recent advances in research on mastocytosis and new information abo ut its pathophysiology, treatment of all categories of mastocytosis continu es to be based primarily on symptom control because no current therapeutic modality is curative.* Because of the heterogeneous nature of disease manif estations in this group of disorders, therapy should be individualized to e ach patient's clinical presentation and prognosis. The mainstay of treatmen t for most categories of mastocytosis are H-1 and H-2 antihistamine blockad e to control anaphylactic symptoms, cutaneous manifestations such as prurit us and flushing, and gastric hypersecretion, and corticosteroids to control malabsorption, ascites, and bone pain and to prevent or ameliorate anaphyl axis.(11, 115-118) Epinephrine is used to treat acute episodes of anaphylax is.(138,159, 172) This article presents the accepted regimens for ameliorat ion of the various mast cell mediator-related symptoms that may be seen in all categories of mastocytosis (Box 1).(114, 117) For patients with more ag gressive forms of disease (categories II-IV),(114) greater understanding of the molecular basis of mastocytosis may afford new treatment strategies. P ast and current approaches in dealing with the three more severe categories of mastocytosis are summarized, and future therapeutic directions for this enigmatic group of diseases are also discussed.