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中文摘要
翻译
除了FIP1L1-PDGFRA融合基因的存在外,关于伊马替尼在临床定义的嗜酸性粒细胞增多综合征(HES)中的反应预测因子知之甚少。
英文摘要
With the exception of the presence of the FIP1L1-PDGFRA fusion gene, little is known about predictors of imatinib response in clinically-defined hypereosinophilic syndrome (HES). In this study, we have stratified HES patients according to their FIP1L1-PDGFRA mutational status and criteria suggestive of a myeloid neoplasm. The peripheral blood and bone marrow specimens were evaluated for dysplastic eosinophils on peripheral smear (abnormal nuclear lobation, uneven granulation, hypogranulation, agranulation), serum B12 level 1000 pg/ml, serum tryptase level 12 ng/mL, anemia and/or thrombocytopenia, bone marrow cellularity >80% with left shift in maturation, dysplastic (spindle-shaped) mast cells on bone marrow biopsy, evidence of reticulin brosis 2+ on bone marrow biopsy, and dysplastic megakaryocytes on bone marrow biopsy. Subjects with FIP1L1-PDGFRA-myeloid neoplasm (FP; n =12), PDGFRA-negative HES with 4 criteria suggestive of a myeloid neoplasm (MHES; n =10), or steroid-refractory PDGFRA-negative HES with <4 myeloid criteria (SR; n = 5) were enrolled in a prospective study of imatinib therapy. The primary outcome was an eosinophil count <1.5 109/L at one month and improvement of clinical symptoms. Clinical, molecular, and bone marrow responses to imatinib were assessed. In addition, a retrospective cohort of 18 subjects with clinically-defined HES who received imatinib (300-400 mg daily 1 month) were classified according to the criteria used in the prospective study. Overall results showed that imatinib response rates were 100% in the FP group (n = 16), 54% in the MHES group (n = 13) and 0% in the SR group (n = 16). The presence of 4 myeloid features was the sole predictor of response. After 18 months in complete remission, imatinib was tapered and discontinued in 8 FP and 1 MHES subjects. Seven subjects (6 FP, 1 MHES) remained in remission off therapy for a median of 29 months (range 14-36). In conclusion, clinical features of MHES predict imatinib response in PDGFRA-negative HES.
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Bone Marrow Histopathological Changes in Neoplastic and Non-Neoplastic Diseases
  • 批准号:
    8565402
  • 项目类别:
  • 资助金额:
    $0.0万
  • 财政年份:
    --
  • 负责人:
    Irina Maric
  • 依托单位:
Laboratory Assessment of Patients with Hypereosinophilic Syndrome
  • 批准号:
    8565378
  • 项目类别:
  • 资助金额:
    $0.0万
  • 财政年份:
    --
  • 负责人:
    Irina Maric
  • 依托单位:
Laboratory Assessment of Patients with Systemic Mastocytosis
  • 批准号:
    10019275
  • 项目类别:
  • 资助金额:
    $0.0万
  • 财政年份:
    --
  • 负责人:
    Irina Maric
  • 依托单位:
Laboratory Assessment of Patients with Hypereosinophilic Syndrome
  • 批准号:
    10684570
  • 项目类别:
  • 资助金额:
    $0.0万
  • 财政年份:
    --
  • 负责人:
    Irina Maric
  • 依托单位:
国内基金
海外基金
基于构建骨骼类器官模型探究Fanconi anemia信号通路调控电刺激诱导神经化成骨过程的机制研究
  • 批准号:
    82302715
  • 项目类别:
    青年科学基金项目
  • 资助金额:
    30万元
  • 批准年份:
    2023
  • 负责人:
    熊泽康
  • 依托单位:
FANCM蛋白在传统Fanconi anemia通路以外对保护基因组稳定性的功能
  • 批准号:
  • 项目类别:
    省市级项目
  • 资助金额:
    10.0万元
  • 批准年份:
    2021
  • 负责人:
    陈英伟
  • 依托单位:
范可尼贫血(Fanconi Anemia)基因FANCM在复制后修复中的作用及FA癌症抑制通路的机制研究
  • 批准号:
    31200592
  • 项目类别:
    青年科学基金项目
  • 资助金额:
    23.0万元
  • 批准年份:
    2012
  • 负责人:
    孙伟力
  • 依托单位: