Characteristics of responders (CR, CRi, PR)
| Patient . | Antec MPN . | Karyotype . | Molecular type (VAF) . | VEN regimen . | VEN dose/induction . | VEN cycles . | Best response . | Cycles to best response . | Response: DoR/outcome . |
|---|---|---|---|---|---|---|---|---|---|
| 69 y/F | PV 3 y (HU), MF 1 y (RUX) | add(21)(p11.2) | JAK2 V617F (89%), IDH2 R140Q (31%), RUNX1 D198G (23%), SRSF2 P95L (10%) | Cladribine-LDAC-VEN | 100 mg [VORI] | 4 | CR, MRD(–) | 1 | 4 mo → SCT, ongoing |
| 46 y/M | ET 19 y, MF 1 y (RUX) | Diploid | CALR L367fs (41%), ASXL1 G646 (35%), EZH2 D185H (39%), SETBP1 D868N (28%), PTPN11 R265Q (<10%), IDH1 R132 (20%) | IDH1i-VEN | 800 mg | 3 | CR, MRD(+) | 1 | 3 mo → SCT, ongoing |
| 74 y/F | MPN-u 4 y (HU) | Monosomy 7 | JAK2 V617F (27%), TP53 G302E (<10%), U2AF1 Q157R (<3%) | DAC-VEN | 200 mg [ISA] | 14 | CR, MRD(–) | 2 | 18 mo, ongoing on RX |
| 73 y/F | MF 1 y (supp) | Diploid | MPL W288 (unk), NPM1 W288 (31%), DNMT3A R882H (<2%) | DAC-VEN | 100 mg [POSA] | 5 | CRp, MRD(–) | 3 | 5 cycles/R (died, infection) |
| 76 y/M | ET 6 y (HU) | +8, +20 | NRAS G12D (12%), U2AF1 Q157P (<3%), TET2 N1890S (42%), PTPN11 R265Q (28%), DNMT3A R882H (<10%) | DAC-VEN | 200 mg [ISA] | 6 | CRp, MRD(+) | 2 | 4 cycles/R (next RX) |
| 65 y/M | PV 22 y (HU) | +1,der(1;7)(q10;p10) | JAK2 V671F (42%), IDH1 R132H (18%), RUNX1 T196I (10%), WT1 E384 (<5%) | DAC-VEN | 200 mg [ISA] | 2 | CRn, MRD(+) | 1 | 1 cycle/R (next RX) |
| 74 y/F | MF 3 y (RUX) | +Y, t(8;21) | JAK2 V617F (55%), TET2 L590¥F (<3%) | DAC-VEN-GO | 100 mg (POSA) | 1 | PR [7% bl] MRD(+) | 1 | 3 wks/died with SDH (palliative) |
| Patient . | Antec MPN . | Karyotype . | Molecular type (VAF) . | VEN regimen . | VEN dose/induction . | VEN cycles . | Best response . | Cycles to best response . | Response: DoR/outcome . |
|---|---|---|---|---|---|---|---|---|---|
| 69 y/F | PV 3 y (HU), MF 1 y (RUX) | add(21)(p11.2) | JAK2 V617F (89%), IDH2 R140Q (31%), RUNX1 D198G (23%), SRSF2 P95L (10%) | Cladribine-LDAC-VEN | 100 mg [VORI] | 4 | CR, MRD(–) | 1 | 4 mo → SCT, ongoing |
| 46 y/M | ET 19 y, MF 1 y (RUX) | Diploid | CALR L367fs (41%), ASXL1 G646 (35%), EZH2 D185H (39%), SETBP1 D868N (28%), PTPN11 R265Q (<10%), IDH1 R132 (20%) | IDH1i-VEN | 800 mg | 3 | CR, MRD(+) | 1 | 3 mo → SCT, ongoing |
| 74 y/F | MPN-u 4 y (HU) | Monosomy 7 | JAK2 V617F (27%), TP53 G302E (<10%), U2AF1 Q157R (<3%) | DAC-VEN | 200 mg [ISA] | 14 | CR, MRD(–) | 2 | 18 mo, ongoing on RX |
| 73 y/F | MF 1 y (supp) | Diploid | MPL W288 (unk), NPM1 W288 (31%), DNMT3A R882H (<2%) | DAC-VEN | 100 mg [POSA] | 5 | CRp, MRD(–) | 3 | 5 cycles/R (died, infection) |
| 76 y/M | ET 6 y (HU) | +8, +20 | NRAS G12D (12%), U2AF1 Q157P (<3%), TET2 N1890S (42%), PTPN11 R265Q (28%), DNMT3A R882H (<10%) | DAC-VEN | 200 mg [ISA] | 6 | CRp, MRD(+) | 2 | 4 cycles/R (next RX) |
| 65 y/M | PV 22 y (HU) | +1,der(1;7)(q10;p10) | JAK2 V671F (42%), IDH1 R132H (18%), RUNX1 T196I (10%), WT1 E384 (<5%) | DAC-VEN | 200 mg [ISA] | 2 | CRn, MRD(+) | 1 | 1 cycle/R (next RX) |
| 74 y/F | MF 3 y (RUX) | +Y, t(8;21) | JAK2 V617F (55%), TET2 L590¥F (<3%) | DAC-VEN-GO | 100 mg (POSA) | 1 | PR [7% bl] MRD(+) | 1 | 3 wks/died with SDH (palliative) |
Antec., antecedent; CRn, complete remission without neutrophil recovery; CRp, complete remission without platelet recovery; DoR, duration of response; F, female; GO, gemtuzumab; HU, hydroxyurea; IDH1-i, IDH1 inhibitor; ISA, isavuconazole; M, male; MF, myelofibrosis; POSA, posaconazole; R, relapsed; RX, therapy; SDH, subdural hematoma; supp; supportive therapy (included steroids and danazol); VAF, variant allele frequency; VORI, voriconazole.