期刊
LEUKEMIA
卷 21, 期 6, 页码 1183-1188出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/sj.leu.2404662
关键词
FIP1L1-PDGFRA; acute myeloid leukemia; lymphoblastic T-NHL
The FIP1L1-PDGFRA fusion gene has been described in patients with eosinophilia-associated myeloproliferative disorders (Eos-MPD). Here, we report on seven FIP1L1-PDGFRA-positive patients who presented with acute myeloid leukemia (AML, n = 5) or lymphoblastic T-cell non-Hodgkin-lymphoma (n = 2) in conjunction with AML or Eos-MPD. All patients were male, the median age was 58 years (range, 40-66). AML patients were negative for common mutations of FLT3, NRAS, NPM1, KIT, MLL and JAK2; one patient revealed a splice mutation of RUNX1 exon 7. Patients were treated with imatinib (100 mg, n = 5; 400 mg, n = 2) either as monotherapy (n = 2), as maintenance treatment after intensive chemotherapy (n = 3) or in overt relapse 43 and 72 months, respectively, after primary diagnosis and treatment of FIP1L1-PDGFRA-positive disease (n = 2). All patients are alive, disease-free and in complete hematologic and complete molecular remission after a median time of 20 months (range, 9-36) on imatinib. The median time to achievement of complete molecular remission was 6 months (range, 1-14). We conclude that all eosinophilia-associated hematological malignancies should be screened for the presence of the FIP1L1-PDGFRA fusion gene as they are excellent candidates for treatment with tyrosine kinase inhibitors even if they present with an aggressive phenotype such as AML.
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