Treatment of mastocytosis: pharmacologic basis and current concepts.
Mastocytosis is a rare, heterogeneous disorder characterized by a marked increase in mast cell density in various tissues. Mast cells from different human tissues are heterogeneous. So far, there is no cure for systemic mastocytosis. Conventional therapy is based on agents that antagonize mediators released from mast cells, drugs that inhibit the release of mediators and agents that modulate mast cell proliferation. This pharmacologic approach is satisfactory in the majority of patients with indolent mastocytosis. At the beginning of the new millennium, the therapy of severe forms of aggressive mastocytosis remains a challenge for students of this intriguing disorder.
Marone
G
G
Divisione di Immunologia Clinica e Allergologia, Università di Napoli Federico II, Facoltà di Medicina, Via S. Pansini 5, 80131 Naples, Italy. marone@unina.it
Spadaro
G
G
Granata
F
F
Triggiani
M
M
D000262:Adenylyl Cyclases / Q000378:metabolism; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000777:Anesthetics / Q000009:adverse effects / Q000494:pharmacology; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000242:Cyclic AMP / Q000502:physiology; D021983:Cyclophilins / Q000037:antagonists & inhibitors; D004789:Enzyme Activation / Q000187:drug effects; D006634:Histamine H1 Antagonists / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018836:Inflammation Mediators / Q000502:physiology; D007371:Interferon-gamma / Q000627:therapeutic use; D008407:Mast Cells / Q000145:classification / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D008415:Mastocytosis / Q000188:drug therapy*; D009928:Organ Specificity; D011701:PUVA Therapy; D015398:Signal Transduction / Q000187:drug effects; D014582:Urticaria Pigmentosa / Q000188:drug therapy
D000305:Adrenal Cortex Hormones; D000777:Anesthetics; D000894:Anti-Inflammatory Agents, Non-Steroidal; D006634:Histamine H1 Antagonists; D007166:Immunosuppressive Agents; D018836:Inflammation Mediators; D007371:Interferon-gamma; D000242:Cyclic AMP; D000262:Adenylyl Cyclases; D021983:Cyclophilins