Imatinib mesylate may induce long-term clinical response in FIP1L1-PDGFRα-negative hypereosinophilic syndrome.
The idiopathic hypereosinophilic syndrome (HES) comprises a heterogenous group of disorders characterized by marked blood eosinophilia with eosinophilia-associated organ damage. Eight patients with a median age at diagnosis of 42 years (range 19-67) received imatinib mesylate (IM) for FIP1L1-PDGFRα-negative HES resistant to previous conventional treatment. Median number of prior therapies was 3 (range 2-4). Median time from diagnosis to IM initiation was 112 months (range 2-293). Four patients were treated daily with 100 mg IM, whereas the remaining four patients were treated daily with 400 mg IM. Four male patients (50%) achieved complete haematologic response (CHR) after median of 7 days (range 3-150) using 100 mg daily IM (n = 2) and 400 mg (n = 2). Median duration of IM treatment for IM responders was 18 months (range 2-88). No adverse events were reported throughout the treatment duration. Two patients maintained CHR while on 100 mg weekly IM. Four patients (2 men and 2 women) failed IM treatment. Median duration of IM for non-responding patients was 3 weeks (range 3-12). Non-responding HES patients were significantly older and had lower percentage of blood eosinophilia when compared with IM responders. Our results suggest that IM, even at lower than conventional doses, may induce and maintain long-term remission for FIP1L1-PDGFRα-negative HES.
Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Dabrowski Street 25, 40-032, Katowice, Poland. ghelbig@o2.pl
Grzegorz
G
Helbig
Marek
M
Hus
Magdalena
M
Hałasz
Marek
M
Dudziński
Agnieszka
A
Więcławek
Małgorzata
M
Stachowicz
Anna
A
Soja
Sławomira
S
Kyrcz-Krzemień
D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001549:Benzamides; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000378:metabolism*; D000068877:Imatinib Mesylate; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000378:metabolism*; D010879:Piperazines / Q000627:therapeutic use*; D011446:Prospective Studies; D011743:Pyrimidines / Q000627:therapeutic use*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000378:metabolism*; D012074:Remission Induction; D016879:Salvage Therapy; D016896:Treatment Outcome; D055815:Young Adult; D039221:mRNA Cleavage and Polyadenylation Factors / Q000378:metabolism*
D000970:Antineoplastic Agents; D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha
N Engl J Med. 2003 Mar 27;348(13):1201-14
12660384
Br J Haematol. 2008 Apr;141(2):200-4
18307562
Haematologica. 2007 Sep;92(9):1173-9
17666373
Br J Haematol. 2007 Jul;138(1):77-81
17555450
Blood. 2004 Apr 15;103(8):2879-91
15070659
Blood. 2009 Oct 29;114(18):3736-41
19692700
Medicine (Baltimore). 1975 Jan;54(1):1-27
1090795
Leuk Res. 2009 Aug;33(8):1127-9
19144405
Leuk Res. 2009 Jun;33(6):837-9
19013640
Br J Haematol. 2008 Dec;143(5):707-15
18950453
Haematologica. 2009 Sep;94(9):1236-41
19734416
Br J Haematol. 2009 Dec;147(5):681-5
19735261