Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/201642
Type: Artigo de periódico
Title: Familial Systemic Mastocytosis With Germline Kit K509i Mutation Is Sensitive To Treatment With Imatinib, Dasatinib And Pkc412.
Author: de Melo Campos, Paula
Machado-Neto, João A
Scopim-Ribeiro, Renata
Visconte, Valeria
Tabarroki, Ali
Duarte, Adriana S S
Barra, Flávia F C
Vassalo, José
Rogers, Heesun J
Lorand-Metze, Irene
Tiu, Ramon V
Costa, Fernando F
Olalla Saad, Sara T
Traina, Fabiola
Abstract: Mastocytosis are myeloproliferative neoplasms commonly related to gain-of-function mutations involving the tyrosine kinase domain of KIT. We herein report a case of familial systemic mastocytosis with the rare KIT K509I germ line mutation affecting two family members: mother and daughter. In vitro treatment with imatinib, dasatinib and PKC412 reduced cell viability of primary mast cells harboring KIT K509I mutation. However, imatinib was more effective in inducing apoptosis of neoplastic mast cells. Both patients with familial systemic mastocytosis had remarkable hematological and skin improvement after three months of imatinib treatment, suggesting that it may be an effective front line therapy for patients harboring KIT K509I mutation.
Subject: Adult
Apoptosis
Base Sequence
Benzamides
Blotting, Western
Female
Germ-line Mutation
Humans
Mastocytosis, Systemic
Piperazines
Protein Kinase Inhibitors
Proto-oncogene Proteins C-kit
Pyrimidines
Staurosporine
Thiazoles
Young Adult
Dasatinib
Familial Mastocytosis
Imatinib
K509i Kit Mutation
Pkc412
Tyrosine Kinase Inhibitors
Rights: fechado
Identifier DOI: 10.1016/j.leukres.2014.07.010
Address: http://www.ncbi.nlm.nih.gov/pubmed/25139846
Date Issue: 2014
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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