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FIP1L1/PDGFR alpha-associated systemic mastocytosis Yamada Y; Cancelas JAInt Arch Allergy Immunol 2010[]; 152 Suppl 1 (Suppl 1): 101-5Since the identification of the FIP1L1/PDGFRA fusion gene as a pathogenic cause of the hypereosinophilic syndrome (HES), the importance of the molecular classification of HES leading to the diagnosis of chronic eosinophilic leukemia (CEL) has been recognized. As a result, a new category, 'myeloid and lymphoid neoplasm with eosinophilia and abnormalities in PDGFRA, PDGFRB or FGFR1', has recently been added to the new WHO criteria for myeloid neoplasms. FIP1L1/PDGFR alpha-positive disorders are characterized by clonal hypereosinophilia, multiple organ dysfunctions due to eosinophil infiltration, systemic mastocytosis (SM) and a dramatic response to treatment with imatinib mesylate. A murine HES/CEL model by the introduction of FIP1L1/PDGFR alpha and IL-5 overexpression also shows SM, representing patients with FIP1L1/PDGFR alpha-positive HES/CEL/SM. The murine model and the in vitro development system of FIP1L1/PDGFR alpha-positive mast cells revealed the interaction between FIP1L1/PDGFR alpha, IL-5 and stem cell factor in the development of HES/CEL/SM. Current findings of FIP1L1/PDGFR alpha-positive HES/CEL are reviewed focusing on aberrant mast cell development leading to SM.|Animals[MESH]|Eosinophilia/etiology/metabolism/pathology[MESH]|Humans[MESH]|Mastocytosis, Systemic/complications/etiology/*metabolism/pathology[MESH]|Oncogene Proteins, Fusion/genetics/*metabolism[MESH]|Receptor, Platelet-Derived Growth Factor alpha/genetics/*metabolism[MESH]|mRNA Cleavage and Polyadenylation Factors/genetics/*metabolism[MESH] |
