The Spectrum of FIP1L1-PDGFRA-Associated Chronic Eosinophilic Leukemia: New Insights Based on a Survey of 44 Cases.

Medicine (Baltimore) 2013 Sep;92(5):e1-e9

From the French Eosinophil Network-EA-2686 and Department of Immunology (FL, SM, DL, SD, LP, JEK); Laboratory of Hematology, INSERM U837-Institut de Recherche contre le Cancer (AR, CP); Department of Internal Medicine (PYH); and INSERM U995 (MC), CHRU de Lille, Université Lille-Nord de France, Lille; CNRS UMR 8147 and Department of Hematology (EM), Hôpital Necker-Enfants Malades Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-5 Descartes, Paris; Department of Internal Medicine (FA, OB, JEK), Hôpital Foch, Université Versailles Saint-Quentin en Yvelines, Suresnes; Laboratory of Hematology (JMC), Hôpital Saint-Louis (AP-HP), Université Paris-7 Diderot, Paris; Department of Hematology (PR), Hôpital André Mignot, Université Versailles Saint-Quentin en Yvelines, Le Chesnay; Department of Hematology (AST), CHU Angers, Angers; Department of Internal Medicine (OF), Hôpital Jean Verdier (AP-HP), Bondy; Department of Internal Medicine (MM), Hôpital Henri Mondor (AP-HP), Créteil; Department of Internal Medicine and Oncology (JPdJ), Hôpital d'Instruction des Armées Sainte-Anne, Toulon; Department of Hematology (PCM), Centre Hospitalier d'Annecy, Pringy; Department of Internal Medicine (DS), Hôpital Lariboisière (AP-HP), Paris; Department of Pneumology (VC), Hôpital Louis Pradel-Hospices Civils de Lyon, Lyon; Department of Internal Medicine A (MH), CHU Hotel-Dieu, Nantes; Department of Internal Medicine (OL), Hôpital Bichat-Claude Bernard (AP-HP), Paris; and Department of Pediatric Hematology (AB), Hôpital Robert Debré (AP-HP), Paris; France.

Imatinib is the treatment of choice for FIP1L1/PDGFRA (F/P)-associated chronic eosinophilic leukemia (F/P CEL), but its optimal dosing, duration, and possibility of discontinuation are still a matter of debate. A retrospective multicenter study was conducted with 44 F/P CEL patients identified in the French Eosinophil Network and treated with imatinib. The most frequently involved systems were skin (57%), spleen (52%), and lung (45%), and eosinophilic heart disease was observed in 15 patients (34%). Complete hematologic response (CHR) was obtained in all patients, and complete molecular response (CMR) in 95% of patients (average initial imatinib dose, 165 mg/d). For 29 patients the imatinib dose was tapered with a maintenance dose of 58 mg/d (±34 mg/d), allowing sustained CHR and CMR. None of the patients developed resistance during a median follow-up of 52.3 months (range, 1.4-97.4 mo). Imatinib was stopped in 11 patients; 6 of the patients subsequently relapsed, but 5 remained in persistent CHR or CMR (range, 9-88 mo). These results confirm that an initial low-dose regimen of imatinib (100 mg/d) followed by a lower maintenance dose can be efficient for obtaining long-term CHR and CMR. Our data also suggest that imatinib can be stopped in some patients without molecular relapse.

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Source
http://dx.doi.org/10.1097/MD.0b013e3182a71ebaDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4553979PMC
September 2013
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