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Blood, 1 January 2007, Vol. 109, No. 1, pp. 61-64.
Prepublished online as a Blood First Edition Paper on September 7, 2006; DOI 10.1182/blood-2006-05-024828.
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CLINICAL TRIALS AND OBSERVATIONS
Brief report
Durable responses to imatinib in patients with PDGFRB fusion gene–positive and BCR-ABL–negative chronic myeloproliferative disorders
Marianna David1,
Nicholas C. P. Cross2,
Sonja Burgstaller3,
Andrew Chase2,
Claire Curtis2,
Raymond Dang4,
Martine Gardembas5,
John M. Goldman6,
Francis Grand2,
George Hughes7,
Francoise Huguet8,
Louise Lavender9,
Grant A. McArthur10,
Francois X. Mahon11,
Giorgio Massimini12,
Junia Melo6,
Philippe Rousselot13,
Robin J. Russell-Jones14,
John F. Seymour10,
Graeme Smith15,
Alastair Stark4,
Katherine Waghorn2,
Zariana Nikolova12, and
Jane F. Apperley6,
1 Department of Haematology, University of Pecs, Pecs, Hungary;
2 Wessex Regional Genetics Laboratory, Salisbury District Hospital, Salisbury, United Kingdom;
3 Department of Internal Medicine, General Hospital, Wels, Austria;
4 Department of Haematology, Dumfries General Infirmary, Dumfries, Scotland, United Kingdom;
5 Department of Haematology, Centre Hospitalier Universitaire (CHU) Angers, Angers, France;
6 Department of Haematology, Faculty of Medicine, Imperial College, London, United Kingdom;
7 Department of Haematology, West Middlesex Hospital, London, United Kingdom;
8 Department of Haematology, Hopital de Purpan, Toulouse, France;
9 Molecular Pathology Unit, Southampton General Hospital, Southampton, United Kingdom;
10 Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Australia;
11 Laboratory of normal and pathological haematology, University Victor Ségalen, Bordeaux, France;
12 Novartis Oncology, Basel, Switzerland;
13 Department of Haematology, Hopital St Louis, Paris, France;
14 Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Road, London, United Kingdom; and
15 Department of Haematology, Leeds General Infirmary, Leeds, United Kingdom
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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