[Treatment of systemic mastocytosis].
BACKGROUND
Systemic mastocytosis is a rare disease, characterized by mast cells proliferation in various organs. Two types of clinical manifestations can be distinguished: those related to mast cells mediators release and those related to tumoral proliferation involving different organs, these later defining aggressive systemic mastocytosis. Until recently, treatment was mainly symptomatic, without anti tumoral effect.
These last years, advances have been made in the understanding of the disease with the discovery of the c-kit oncogene mutation and the approach of the disease as a myeloproliferative disorder.


CONCLUSIONS
Based on experiences acquired in the treatment of this kind of disorders, evaluation of new therapeutics, such as cladribine or combination of interferon-alpha and cytarabine is in progress. At least, tyrosine kinase inhibitors, a new family of molecules, are able of inhibiting some types of the mutated c-kit protein and one of them, imatinib mesylate, has shown a great efficacy in the treatment of gastro intestinal stromal tumors (GIST) which also involves the c-kit mutation. By analogy, treatment of patients with c-kit susceptible mutation might be treated with this molecule.
Marrache
F
F
Fédération de médecine interne, maladies infectieuses et tropicales, hôpital Avicenne, université Paris-Nord, 125, route de Stalingrad, 93009 Bobigny, France.
Mémain
N
N
Bonté
I
I
Barete
S
S
Casassus
P
P
de Gennes
C
C
Fain
O
O
Hermine
O
O
Lortholary
O
O
D000305:Adrenal Cortex Hormones / Q000494:pharmacology; D000970:Antineoplastic Agents / Q000494:pharmacology*; D001549:Benzamides; D017338:Cladribine / Q000494:pharmacology*; D003561:Cytarabine / Q000494:pharmacology*; D004164:Diphosphonates / Q000494:pharmacology; D004791:Enzyme Inhibitors / Q000494:pharmacology*; D006634:Histamine H1 Antagonists / Q000494:pharmacology; D006801:Humans; D000068877:Imatinib Mesylate; D016898:Interferon-alpha / Q000494:pharmacology*; D034721:Mastocytosis, Systemic / Q000188:drug therapy* / Q000503:physiopathology / Q000532:radiotherapy; D010778:Photochemotherapy; D010879:Piperazines / Q000494:pharmacology*; D019009:Proto-Oncogene Proteins c-kit / Q000494:pharmacology*; D011743:Pyrimidines / Q000494:pharmacology*
D000305:Adrenal Cortex Hormones; D000970:Antineoplastic Agents; D001549:Benzamides; D004164:Diphosphonates; D004791:Enzyme Inhibitors; D006634:Histamine H1 Antagonists; D016898:Interferon-alpha; D010879:Piperazines; D011743:Pyrimidines; D003561:Cytarabine; D017338:Cladribine; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit
Traitement des mastocytoses systémiques.