TNF alpha promotes clonal dominance of KIT D816V+ cells in mastocytosis: role of survivin and impact on prognosis

Systemic mastocytosis (SM) is defined by expansion and accumulation of neoplastic mast cells (MC) in the bone marrow (BM) and extracutaneous organs. Most patients harbor a somatic KIT D816V mutation, which leads to growth factor independent KIT activation and accumulation of MC. Tumor necrosis factor-alpha (TNF) is a pro-apoptotic and inflammatory cytokine that has been implicated in clonal selection of neoplastic cells. We found that KIT D816V increases expression and secretion of TNF. …