[Systemic mastocytosis].
Systemic mastocytosis is characterized by mast cell proliferation in different organs. Classification delineates 4 categories: indolent systemic mastocytosis, systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease, aggressive systemic mastocytosis and mast cell leukaemia. Clinical manifestations are due to organ infiltration (skin, bone, gut, liver, spleen, lymph nodes) and release of mast-cell mediators. Diagnosis of mastocytosis is based on appropriate stains (Giemsa, Toluidine) and immunophenotype features (tryptase, CD117). Serum level of tryptase reflects the total burden of mast cells. Treatment must prevent mast cell mediators release (histamine antagonists, cromolyn sodium, corticosteroids, leukotriene-receptor inhibitors) and have a cytoreductive effect (interferon, cladribine, tyrosine kinase inhibitors).
Fain
Olivier
O
Service de médecine interne, hôpital Jean Verdier, Université Paris XIII, 93140 Bondy. olivier.fain@jvr.aphp.fr
Stirnemann
Jerôme
J
Lortholary
Olivier
O
D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D006801:Humans; D016130:Immunophenotyping; D034721:Mastocytosis, Systemic / Q000145:classification* / Q000150:complications / Q000276:immunology / Q000473:pathology*; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors
D004791:Enzyme Inhibitors; D011505:Protein-Tyrosine Kinases