Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders

David, M., Cross, N.C., Burgstaller, S., Chase, A., Curtis, C., Dang, R., Gardembas, M., Goldman, J.M.F., Hughes, G., Huguet, F., Lavender, L., McArthur, G.A., Mahon, F.X., Massimini, G., Melo, J., Rousselot, P., Russell-Jones, R.J., Seymour, J.F., Smith, G., Stark, A., Waghorn, K., Nikolova, Z. and Apperley, J.F. (2007) Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders. Blood, 109 (1), 61-64. (doi:10.1182/blood-2006-05-024828.).

Abstract

Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL-negative chronic myeloproliferative disorders (CMPDs). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL-negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.1-60 months). Eleven had prompt responses with normalization of peripheral-blood cell counts and disappearance of eosinophilia; 10 had complete resolution of cytogenetic abnormalities and decrease or disappearance of fusion transcripts as measured by reverse transcriptase-polymerase chain reaction (RT-PCR). Updates were sought from 8 further patients previously described in the literature; prompt responses were described in 7 and persist in 6. Our data show that durable hematologic and cytogenetic responses are achieved with imatinib in patients with PDGFRB fusion-positive, BCR-ABL-negative CMPDs

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Contributors

Author: N.C. Cross

Author: A. Chase

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