Table 3.

Published studies of MN-eos with PDGFRA or PDGFRB rearrangements treated with imatinib mesylate

Study	No. Patients	Gene rearrangement	Dose	Duration of Therapy	Response
Klion et al.56	6	FIP1L1-PDGFRA	100–400 mg/day	1–12 months	CHR 100% CMR 5/6 (83%)
Metzgeroth et al.57	16	FIP1L1-PDGFRA		12 months	CHR 100% CMR 14/16 (87%)
Helbig G et al.58	6	FIP1L1-PDGFRA	100–400 mg/day *	Median, 30 months	CHR 100% CMR 5/6 (83%)
Legrand F et al.35,§	44	FIP1L1-PDGFRA	Mean, 165 mg/day induction/58 mg/day maintenance	Median, 52 months	CHR 100% CMR 43/44 (95%)
Baccarani et al.59	27	FIP1L1-PDGFRA	100 mg/day escalated to 400 mg/day	Median, 1 month	CHR 100% CMR 100%
Jovanovic et al.60,§	11	FIP1L1-PDGFRA	100–400 mg/day	12 months	CMR 9/11 (82%)
Arefi et al.61,§	8	FIP1L1-PDGFRA	100 mg/day	NR	CHR 100% MRg 100%
David et al.62,†	20	10 ETV6-PDGFRB 7 various PDGFRB fusion partner 3 unknown PDGFRB fusion partner	200–800 mg/day	0.1–60 months	CHR 16/20 (80%) CCyR 15/20 (75%) CMR: 4/8 (50%) ETV6- PDGFRB, 1/8 (12%) other rearrangements
Cheah et al.63,‡	26	18 ETV6-PDGFRB 8 various PDGFRB fusion partners	100–400 mg/day	Median, 10.2 years	OR 96% CCyR 52% CMR 32%

^*Patients were transitioned to once-weekly dosing after achieving remission;

^§Multicenter retrospective study;

^†Multicenter, prospective study + review and update of data from the literature;

^‡Multicenter retrospective study, including data from David M, Cross NC, Burgstaller S, et al. Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders. Blood. 2007;109(1):61–64.

Abbreviations: CHR, complete hematologic response, defined as resolution of symptoms and normalization of blood counts; CMR, complete molecular response defined, as no detectable transcript by RT-PCR; CCyR, complete cytogenetic remission, defined as normal karyptype; OR, objective response.