title: Murine models of eosinophilic leukemia: a model of FIP1L1-PDGFRα initiated chronic eosinophilic leukemia/systemic mastocytosis.
abstract: Chronic eosinophilic leukemia (CEL) was distinguished from hypereosinophilic syndrome (HES) in the 2001 World Health Organization (WHO) criteria. Subsequently, the FIP1L1-PDGFRα (F/P) fusion tyrosine kinase was identified in patients with HES and found to be the most common clonal defect in CEL and the second most frequent mutation in systemic mastocytosis (SM). Introduction of F/P into bone marrow hematopoietic stem cells and progenitors has been used to establish murine models of F/P-myeloproliferative neoplasm and F/P-CEL. IL-5 overexpression and introduction of F/P is required to develop murine CEL. This F/P-CEL model is thought to be an accurate model of the clinical disease. Here we describe the method of F/P-CEL/SM model development and assessment.
authors.affiliation: Division of Allergy and Immunology, Gunma Children's Medical Center, 779 Shimohakoda Hokkitsu, Shibukawa, Gunma, 377-8577, Japan, yamaday@gcmc.pref.gunma.jp.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Yamada
authors.forename: Jose A
authors.initials: JA
authors.lastname: Cancelas
authors.forename: Marc E
authors.initials: ME
authors.lastname: Rothenberg
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006412:Hematopoietic Stem Cells / Q000166:cytology; D017681:Hypereosinophilic Syndrome / Q000276:immunology* / Q000378:metabolism*; D007938:Leukemia; D034721:Mastocytosis, Systemic / Q000276:immunology* / Q000378:metabolism*; D051379:Mice; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000378:metabolism
chemical_list: D020796:Receptor, Platelet-Derived Growth Factor alpha