Details

Autor(en) / Beteiligte

• METZGEROTH, G
• WALZ, C
• MÜLLER, M. C
• BENEKE, H
• MÜLLER, L
• DEL VALLE, F
• AULITZKY, W. E
• WITTKOWSKY, G
• SCHMITZ, N
• SCHULTE, C
• MULLER-HERMELINK, K
• HODGES, E
• SCORE, J
• WHITTAKER, S. J
• DIECKER, F
• DÖHNER, H
• SCHULD, P
• HEHLMANN, R
• HOCHHAUS, A
• CROSS, N. C. P
• REITER, A
• SIEBERT, R
• SCHNITTGER, S
• HAFERLACH, C
• POPP, H
• HAFERLACH, T
• ERBEN, P
• MIX, J

Titel

Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma

Ist Teil von

• Leukemia, 2007-06, Vol.21 (6), p.1183-1188

Ort / Verlag

London: Nature Publishing

Erscheinungsjahr

2007

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.