論文 - 詳細
| RRC ID | 2426 |
|---|---|
| 著者 | Miyazawa K, Kakazu N, Ohyashiki K. |
| タイトル | Clinical features of hypereosinophilic syndrome: FIP1L1-PDGFRA fusion gene-positive disease is a distinct clinical entity with myeloproliferative features and a poor response to corticosteroid. |
| ジャーナル | Int J Hematol |
| Abstract |
We conducted a retrospective analysis of the clinical features of 20 patients with severe eosinophilia at our institution, including 10 cases of hypereosinophilic syndrome (HES) (5 definite and 5 probable cases) and 10 cases of other eosinophilic disorders. Of the 20 patients, 14 initially received prednisolone treatment, which resulted in rapid improvement and normalization of eosinophilia within 8 weeks; however, 2 patients with splenomegaly showed poor control of eosinophilia in response to corticosteroid treatment. In addition, the FIP1L1-PDGFRA fusion gene was detected only in these 2 cases. One of the FIP1L1-PDGFRA - positive HES cases featured bone marrow fibrosis. Treatment of this patient with imatinib mesylate resulted in a dramatic improvement of eosinophilia, organomegaly, and the bone marrow fibrosis. Taken together, our data and previous reports suggest that FIP1L1-PDGFRA - positive HES is a distinct clinical entity with myeloproliferative features and showing a poor response to corticosteroid treatment. |
| 巻・号 | 85(1) |
| ページ | 5-10 |
| 公開日 | 2007-1-1 |
| DOI | 10.1532/IJH97.06047 |
| PII | 11N5520176R65277 |
| PMID | 17261495 |
| MeSH | Adrenal Cortex Hormones / therapeutic use* Adult Aged Bone Marrow / pathology Eosinophilia / drug therapy Eosinophilia / genetics Eosinophilia / pathology Female Fibrosis / etiology Humans Hypereosinophilic Syndrome / drug therapy Hypereosinophilic Syndrome / genetics* Hypereosinophilic Syndrome / pathology* In Situ Hybridization, Fluorescence Male Middle Aged Myeloproliferative Disorders / genetics* Oncogene Proteins, Fusion / genetics* Prednisolone / therapeutic use Prognosis Receptor, Platelet-Derived Growth Factor alpha / genetics* Retrospective Studies Splenomegaly mRNA Cleavage and Polyadenylation Factors / genetics* |
| IF | 2.245 |
| 引用数 | 5 |
| WOS 分野 | HEMATOLOGY |
| リソース情報 | |
| ヒト・動物細胞 | |