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. 2021 Jan 13;23(2):16. doi: 10.1007/s11912-020-01009-3

Table 2.

New therapeutics in pediatric AML

Drug Mechanism of action Current stage of development in pediatric AML
Drug-antibody conjugates
  Gemtuzumab ozogamicin [14] Anti-CD33 antibody with calicheamicin payload

FDA-approved for newly diagnosed AML > 1 month of age

Standard of care as single dose in induction 1

  Flotetuzumab [58] CD123/CD3 bispecific dual-affinity retargeting antibody Phase I trials for r/r AML
  Nivolumab [61] Anti-PD-1 antibody/checkpoint inhibitor Phase I/II trial for r/r AML combined with azacitidine
Epigenetic modifiers
  Decitabine [67] Hypomethylating agent: inhibits DNA methyltransferases

Completed phase I trial for r/r AML

Phase II trial with standard chemotherapy in newly diagnosed AML

  Azacitidine [68] Hypomethylating agent: inhibits DNA methyltransferases

Phase I/II trials for r/r AML

Phase II trial with standard chemotherapy in newly diagnosed AML

  Vorinostat [72] Inhibits histone deacetylase Phase I trial for r/r AML
  Panobinostat [71] Inhibits histone deacetylase Phase I trial for r/r AML
Tyrosine kinase/FLT3 inhibitors
  Sorafenib [80] 1st-generation type II TKI: active against FLT3-ITD and FLT3-TKD mutations Completed phase III trials for high AR FLT3-ITD AML
  Midostaurin [83] 1st-generation type I TKI: active against FLT3 and KIT mutations

USA: phase I/II trials terminated for low enrollment

International: ongoing phase II trial

  Gilteritinib [87] 2nd-generation type I TKI: active against FLT3 and AXL

Phase III trials combined with standard chemo in newly diagnosed AML

Phase I/II trial combined with FLAG for r/r AML

  Quizartinib [75] Type II TKI Phase I/II trials in r/r AML
  Crenolanib [75] Type I TKI Completed phase I trial in r/r leukemias
Others
  Venetoclax [99] Inhibits BCL-2 Phase I/II trials for r/r AML
  CPX-351 [99] Liposomal formulation of daunorubicin and cytarabine—enhances synergy between drugs and extends half-life

Completed phase I/II trials for r/r AML

Current phase III trial for newly diagnosed AML

  Atovaquone [99] Inhibits oxidative phosphorylation and STAT3 activation Phase I trial with standard chemotherapy
Chimeric antigen receptor T cells
  CD123-targeting CAR-T [62] T cells genetically modified to target/kill CD123-expressing AML cells Phase I trials for r/r AML
  CD33-targeting CAR-T [46] T cells genetically modified to target/kill CD33-expressing AML cells Phase I trials for r/r AML

FDA, Food and Drug Administration; r/r, relapsed/refractory; TKI, tyrosine kinase inhibitor; ITD, internal tandem duplication; TKD, tyrosine kinase domain; AR, allelic ratio; FLAG, fludarabine, cytarabine, granulocyte colony-stimulating factor; CAR-T, chimeric antigen receptor T cell