FIP1L1/PDGFRalpha's Kit to stimulate mast cells

摘要

Five years ago, the FIP1L1 /PDGFRA fusion gene was identified in patients with hypereosinophilic syndrome (HES). Since then, many other studies have confirmed the presence of the FIP1L1/PDGFRA fusion gene in a significant fraction of hypereosino-philia patients and have shown that these patients have excellent responses to imatinib treatment. Given the documented clonality of the disease in HES patients, the preferred nomenclature for this disease is FIP1L1 / PDGFRA chronic eosinophilic leukemia (CEL). Eosinophilia is also frequently observed in systemic mastocytosis, and up to half of these cases are also positive for the FIP1L1/ PDGFRA fusion gene. It is currently unde-cided whether to call this disease systemic mastocytosis with eosinophilia, or rather FIP1L1 /PDGFRA-positive CEL, illustrating the close relationship between both FIP1L1 /PDGFRA-positive diseases