Abstract |
Hypereosinophilic syndrome (HES) includes heterogeneous hematological disorders that are characterized by distinctive blood and tissue eosinophilia. In addition to classical HES criteria, the World Health Organization proposed a set of criteria that distinguish chronic eosinophilic leukemia (CEL) from HES. As such, the fusion gene FIP1L1/PDGFRA was found as a cause of CEL in a significant proportion of patients initially diagnosed as having HES. Several investigations have tried to dissect the mechanism of leukemogenesis; eosinophilia and signaling induced by FIP1L1/ PDGFRalpha in cell lines, bone marrow mast cells, primary human eosinophils and in murine myeloproliferative disorder models. In this review, we introduce the current knowledge on the relationship between FIP1L1/ PDGFRalpha and cell signaling, eosinophil proliferation, survival and activation and mastocytosis specially focusing on the evidence learned from murine models.
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Authors | Yoshiyuki Yamada, Jose A Cancelas, Marc E Rothenberg |
Journal | International archives of allergy and immunology
(Int Arch Allergy Immunol)
Vol. 149 Suppl 1
Pg. 102-7
( 2009)
ISSN: 1423-0097 [Electronic] Switzerland |
PMID | 19494514
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Copyright | Copyright 2009 S. Karger AG, Basel. |
Chemical References |
- Benzamides
- Oncogene Proteins, Fusion
- Piperazines
- Protein Kinase Inhibitors
- Pyrimidines
- mRNA Cleavage and Polyadenylation Factors
- Imatinib Mesylate
- FIP1L1-PDGFRA fusion protein, human
- Receptor, Platelet-Derived Growth Factor alpha
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Topics |
- Animals
- Benzamides
- Chronic Disease
- Disease Models, Animal
- Eosinophils
(immunology)
- Humans
- Hypereosinophilic Syndrome
(complications, diagnosis, drug therapy, metabolism)
- Imatinib Mesylate
- Mastocytosis
(etiology, metabolism)
- Mice
- Oncogene Proteins, Fusion
(chemistry, genetics)
- Piperazines
(therapeutic use)
- Protein Kinase Inhibitors
(therapeutic use)
- Pyrimidines
(therapeutic use)
- Receptor, Platelet-Derived Growth Factor alpha
(chemistry, genetics)
- Signal Transduction
- mRNA Cleavage and Polyadenylation Factors
(chemistry, genetics)
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