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- Title
Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma.
- Authors
Metzgeroth, G; Walz, C; Score, J; Siebert, R; Schnittger, S; Haferlach, C; Popp, H; Haferlach, T; Erben, P; Mix, J; Müller, M C; Beneke, H; Müller, L; Del Valle, F; Aulitzky, W E; Wittkowsky, G; Schmitz, N; Schulte, C; Müller-Hermelink, K; Hodges, E; Whittaker, S J; Diecker, F; Döhner, H; Schuld, P; Hehlmann, R; Hochhaus, A; Cross, N C P; Reiter, A
- Abstract
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.
- Publication
Leukemia, 2007, Vol 21, Issue 6, p1183
- ISSN
0887-6924
- Publication type
Journal Article
- DOI
10.1038/sj.leu.2404662