Table 2.

Current VEN-based combination regimens in AML

All molecular subgroupsCycle 1Cycle 2+Comments
Azacitidine+
venetoclax 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-28 
Same as C1 Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support appropriate once response. 
LDAC+
venetoclax 
LDAC 20  mg/m2 subQ daily; days 1-10
Venetoclax 600  mg daily; days 1-28 
Same as C1 Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support appropriate once response 
Cladribine+
LDAC+
venetoclax
(NCT 03586609) 
Cladribine 5  mg/m2 daily; days 1-5
LDAC 20  mg subQ
twice daily; days 1-10
Venetoclax 400 mg
daily; days 1-21 
Alternating cycles of cladribine+LDAC+venetoclax and HMA+venetoclax every 2 cyclesa Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support once response. 
All molecular subgroupsCycle 1Cycle 2+Comments
Azacitidine+
venetoclax 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-28 
Same as C1 Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support appropriate once response. 
LDAC+
venetoclax 
LDAC 20  mg/m2 subQ daily; days 1-10
Venetoclax 600  mg daily; days 1-28 
Same as C1 Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support appropriate once response 
Cladribine+
LDAC+
venetoclax
(NCT 03586609) 
Cladribine 5  mg/m2 daily; days 1-5
LDAC 20  mg subQ
twice daily; days 1-10
Venetoclax 400 mg
daily; days 1-21 
Alternating cycles of cladribine+LDAC+venetoclax and HMA+venetoclax every 2 cyclesa Obtain day 21-28 bone marrow biopsy. If CR/CRi/aplasia, hold therapy and consider reduction of venetoclax duration for cycle 2 onward. Use of G-CSF for ANC support once response. 
IDH1-mutated AMLCycle 1Cycle 2+Comments
Azacitidine+
ivosidenib
 
Azacitidine 75  mg/m2 daily; days 1-7
Ivosidenib 500  mg daily; days 1-14 
Same as C1 Monitor for QT prolongation, IDH-DS. 
Azacitidine+
venetoclax+
ivosidenib
(NCT03471260) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-14
Ivosidenib 500  mg
daily; days 15-28 
Azacitidine 75  mg/m2
daily; days 1-7
Venetoclax 400  mg
daily; days 1-14
Ivosidenib 500  mg
daily; days 1-28 
Currently in phase 2 expansion studies. 
Decitabine/cedazuridine (DEC-C)+
venetoclax+
ivosidenib
(NCT04774393) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 600  mg daily; days 1-14
Ivosidenib 500  mg
daily; days 8-28 
DEC-C 35  mg/100  mg
daily; days 1-7
Venetoclax 600  mg
daily; days 1-14
Ivosidenib 500  mg
daily; days 1-28 
Currently in phase 2 expansion studies. Venetoclax 600 mg selected for phase 2 due to Cyp inducing properties of IVO. Monitor for IDH-DS, QT prolongation with ivosidenib. 
IDH1-mutated AMLCycle 1Cycle 2+Comments
Azacitidine+
ivosidenib
 
Azacitidine 75  mg/m2 daily; days 1-7
Ivosidenib 500  mg daily; days 1-14 
Same as C1 Monitor for QT prolongation, IDH-DS. 
Azacitidine+
venetoclax+
ivosidenib
(NCT03471260) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-14
Ivosidenib 500  mg
daily; days 15-28 
Azacitidine 75  mg/m2
daily; days 1-7
Venetoclax 400  mg
daily; days 1-14
Ivosidenib 500  mg
daily; days 1-28 
Currently in phase 2 expansion studies. 
Decitabine/cedazuridine (DEC-C)+
venetoclax+
ivosidenib
(NCT04774393) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 600  mg daily; days 1-14
Ivosidenib 500  mg
daily; days 8-28 
DEC-C 35  mg/100  mg
daily; days 1-7
Venetoclax 600  mg
daily; days 1-14
Ivosidenib 500  mg
daily; days 1-28 
Currently in phase 2 expansion studies. Venetoclax 600 mg selected for phase 2 due to Cyp inducing properties of IVO. Monitor for IDH-DS, QT prolongation with ivosidenib. 
IDH2-mutated AMLCycle 1Cycle 2+Comments
Decitabine/cedazuridine (DEC-C)+
venetoclax+
enasidenib
(NCT04774393) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg daily; days 1-14
Enasidenib 100  mg
daily; days 8-28 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Enasidenib 100 mg
daily; days 1-28 
Currently in phase 2 expansion studies. Monitor for IDH-DS, hyperbilirubinemia with enasidenib. 
IDH2-mutated AMLCycle 1Cycle 2+Comments
Decitabine/cedazuridine (DEC-C)+
venetoclax+
enasidenib
(NCT04774393) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg daily; days 1-14
Enasidenib 100  mg
daily; days 8-28 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Enasidenib 100 mg
daily; days 1-28 
Currently in phase 2 expansion studies. Monitor for IDH-DS, hyperbilirubinemia with enasidenib. 
FLT3-mutated AMLCycle 1Cycle 2+Comments
FLT3-ITD/TKD
Azacitidine+
venetoclax+
gilteritinib
(NCT04140487) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-14
Gilteritinib 80  mg
daily; days 1-28 
Azacitidine 75  mg/m2
days 1-5
Venetoclax 400  mg
days 1-7
Gilteritinib 80  mg
days 1-28 
Currently in dose-ranging and expansion studies (NCT05520567). Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation and differentiation syndrome with gilteritinib. 
FLT3-ITD only
Decitabine+
venetoclax+
quizartinib
(NCT03661307) 
Decitabine 20  mg/m2 daily; days 1-10
Venetoclax 400  mg daily; days 1-21
Quizartinib 30-40  mg
daily days 1-14 
Decitabine 20  mg/m2
daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Quizartinib 30-40  mg
daily; days 1-28 
Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation with quizartinib. 
FLT3-mutated AML
(R/R)
Venetoclax+
gilteritinib
(NCT03625505) 
Venetoclax 400  mg daily; days 1-28
Gilteritinib 120  mg
daily; days 1-28 
Venetoclax 400  mg
daily; days 1-28
Gilteritinib 120 mg
daily; days 1-28 
Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation and differentiation syndrome with gilteritinib. 
FLT3-mutated AMLCycle 1Cycle 2+Comments
FLT3-ITD/TKD
Azacitidine+
venetoclax+
gilteritinib
(NCT04140487) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-14
Gilteritinib 80  mg
daily; days 1-28 
Azacitidine 75  mg/m2
days 1-5
Venetoclax 400  mg
days 1-7
Gilteritinib 80  mg
days 1-28 
Currently in dose-ranging and expansion studies (NCT05520567). Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation and differentiation syndrome with gilteritinib. 
FLT3-ITD only
Decitabine+
venetoclax+
quizartinib
(NCT03661307) 
Decitabine 20  mg/m2 daily; days 1-10
Venetoclax 400  mg daily; days 1-21
Quizartinib 30-40  mg
daily days 1-14 
Decitabine 20  mg/m2
daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Quizartinib 30-40  mg
daily; days 1-28 
Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation with quizartinib. 
FLT3-mutated AML
(R/R)
Venetoclax+
gilteritinib
(NCT03625505) 
Venetoclax 400  mg daily; days 1-28
Gilteritinib 120  mg
daily; days 1-28 
Venetoclax 400  mg
daily; days 1-28
Gilteritinib 120 mg
daily; days 1-28 
Perform bone marrow examination on C1D14 and interrupt therapy if CR/CRi or aplasia obtained to permit hematopoietic recovery. Monitor for QT prolongation and differentiation syndrome with gilteritinib. 
NPM1/KMT2Ar AMLCycle 1Cycle 2+Comments
Azacitidine+
venetoclax+
revumenib (REV)
(NCT03013998) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-28
Revumenib 113/163  mg
Every 12 hours; days 1-28 
Same as C1 Randomized expansion cohorts of dose level 1a (REV 113  mg) and dose level 2a (REV 163 mg) are ongoing to determine optimal dosing in triplet combination. 
(R/R)
Decitabine-cedazuridine (DEC-C)+
venetoclax+
revumenib
(NCT05360160) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg daily; days 1-14
Revumenib 113  mg
Q12H; days 1-28 
DEC-C 35  mg/100  mg
daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Revumenib
Q12H; days 1-28 
Phase 1b dose-finding cohort enrollment ongoing. Monitor for differentiation syndrome associated with menin inhibitors. 
NPM1/KMT2Ar AMLCycle 1Cycle 2+Comments
Azacitidine+
venetoclax+
revumenib (REV)
(NCT03013998) 
Azacitidine 75  mg/m2 daily; days 1-7
Venetoclax 400  mg daily; days 1-28
Revumenib 113/163  mg
Every 12 hours; days 1-28 
Same as C1 Randomized expansion cohorts of dose level 1a (REV 113  mg) and dose level 2a (REV 163 mg) are ongoing to determine optimal dosing in triplet combination. 
(R/R)
Decitabine-cedazuridine (DEC-C)+
venetoclax+
revumenib
(NCT05360160) 
DEC-C 35  mg/100  mg daily; days 1-5
Venetoclax 400  mg daily; days 1-14
Revumenib 113  mg
Q12H; days 1-28 
DEC-C 35  mg/100  mg
daily; days 1-5
Venetoclax 400  mg
daily; days 1-14
Revumenib
Q12H; days 1-28 
Phase 1b dose-finding cohort enrollment ongoing. Monitor for differentiation syndrome associated with menin inhibitors. 
a

Consolidation therapy for cladribine+LDAC+venetoclax for cycle 2 onward was as follows: cycle 2 consisted of cladribine 5  mg/m2 on days 1 to 3, LDAC 20  mg subQ twice daily on days 1 to 10, and venetoclax 400  mg on days 1 to 21. Cycle 3 and 4 consisted of azacitidine 75  mg/m2 on days 1 to 7 with venetoclax 400  mg on days 1 to 21. Treatment would then alternate every 2 cycles between the triplet and doublet regimens (the duration of venetoclax was modified for patients in CR/CRi and based on MRD status).

ANC, absolute neutrophil count; G-CSF, granulocyte colony stimulating factor; R/R, relapsed/refractory AML.

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