Acute Myeloid Leukemia, Version 3.2023, NCCN Clinical Practice Guidelines in Oncology

Authors:
Daniel A. Pollyea University of Colorado Cancer Center

Search for other papers by Daniel A. Pollyea in
Current site
Google Scholar
PubMed
Close
 MD, MS
,
Jessica K. Altman Robert H. Lurie Comprehensive Cancer Center of Northwestern University

Search for other papers by Jessica K. Altman in
Current site
Google Scholar
PubMed
Close
 MD
,
Rita Assi Indiana University Melvin and Bren Simon Comprehensive Cancer Center

Search for other papers by Rita Assi in
Current site
Google Scholar
PubMed
Close
 MD
,
Dale Bixby University of Michigan Rogel Cancer Center

Search for other papers by Dale Bixby in
Current site
Google Scholar
PubMed
Close
 MD, PhD
,
Amir T. Fathi Massachusetts General Hospital Cancer Center

Search for other papers by Amir T. Fathi in
Current site
Google Scholar
PubMed
Close
 MD
,
James M. Foran Mayo Clinic Comprehensive Cancer Center

Search for other papers by James M. Foran in
Current site
Google Scholar
PubMed
Close
 MD
,
Ivana Gojo The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins

Search for other papers by Ivana Gojo in
Current site
Google Scholar
PubMed
Close
 MD
,
Aric C. Hall University of Wisconsin Carbone Cancer Center

Search for other papers by Aric C. Hall in
Current site
Google Scholar
PubMed
Close
 MD
,
Brian A. Jonas UC Davis Comprehensive Cancer Center

Search for other papers by Brian A. Jonas in
Current site
Google Scholar
PubMed
Close
 MD, PhD
,
Ashwin Kishtagari Vanderbilt-Ingram Cancer Center

Search for other papers by Ashwin Kishtagari in
Current site
Google Scholar
PubMed
Close
 MD
,
Jeffrey Lancet Moffitt Cancer Center

Search for other papers by Jeffrey Lancet in
Current site
Google Scholar
PubMed
Close
 MD
,
Lori Maness Fred & Pamela Buffett Cancer Center

Search for other papers by Lori Maness in
Current site
Google Scholar
PubMed
Close
 MD
,
James Mangan UC San Diego Moores Cancer Center

Search for other papers by James Mangan in
Current site
Google Scholar
PubMed
Close
 MD, PhD
,
Gabriel Mannis Stanford Cancer Institute

Search for other papers by Gabriel Mannis in
Current site
Google Scholar
PubMed
Close
 MD
,
Guido Marcucci City of Hope National Medical Center

Search for other papers by Guido Marcucci in
Current site
Google Scholar
PubMed
Close
 MD
,
Alice Mims The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute

Search for other papers by Alice Mims in
Current site
Google Scholar
PubMed
Close
 MD, MS
,
Kelsey Moriarty UT Southwestern Simmons Comprehensive Cancer Center

Search for other papers by Kelsey Moriarty in
Current site
Google Scholar
PubMed
Close
 MS, CGC
,
Moaath Mustafa Ali Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute

Search for other papers by Moaath Mustafa Ali in
Current site
Google Scholar
PubMed
Close
 MD, MPH
,
Jadee Neff Duke Cancer Institute

Search for other papers by Jadee Neff in
Current site
Google Scholar
PubMed
Close
 MD, PhD
,
Reza Nejati Fox Chase Cancer Center

Search for other papers by Reza Nejati in
Current site
Google Scholar
PubMed
Close
 MD
,
Rebecca Olin UCSF Helen Diller Family Comprehensive Cancer Center

Search for other papers by Rebecca Olin in
Current site
Google Scholar
PubMed
Close
 MD, MS
,
Mary-Elizabeth Percival Fred Hutchinson Cancer Center

Search for other papers by Mary-Elizabeth Percival in
Current site
Google Scholar
PubMed
Close
 MD, MS
,
Alexander Perl Abramson Cancer Center at the University of Pennsylvania

Search for other papers by Alexander Perl in
Current site
Google Scholar
PubMed
Close
 MD
,
Amanda Przespolewski Roswell Park Comprehensive Cancer Center

Search for other papers by Amanda Przespolewski in
Current site
Google Scholar
PubMed
Close
 DO
,
Dinesh Rao UCLA Jonsson Comprehensive Cancer Center

Search for other papers by Dinesh Rao in
Current site
Google Scholar
PubMed
Close
 MD, PhD
,
Farhad Ravandi The University of Texas MD Anderson Cancer Center

Search for other papers by Farhad Ravandi in
Current site
Google Scholar
PubMed
Close
 MD
,
Rory Shallis Yale Cancer Center/Smilow Cancer Hospital

Search for other papers by Rory Shallis in
Current site
Google Scholar
PubMed
Close
 MD
,
Paul J. Shami Huntsman Cancer Institute at the University of Utah

Search for other papers by Paul J. Shami in
Current site
Google Scholar
PubMed
Close
 MD
,
Eytan Stein Memorial Sloan Kettering Cancer Center

Search for other papers by Eytan Stein in
Current site
Google Scholar
PubMed
Close
 MD
,
Richard M. Stone Dana-Farber/Brigham and Women’s Cancer Center

Search for other papers by Richard M. Stone in
Current site
Google Scholar
PubMed
Close
 MD
,
Kendra Sweet Moffitt Cancer Center

Search for other papers by Kendra Sweet in
Current site
Google Scholar
PubMed
Close
 MD, MS
,
Swapna Thota St. Jude Children’s Research Hospital/The University of Tennessee Health Science Center

Search for other papers by Swapna Thota in
Current site
Google Scholar
PubMed
Close
 MD
,
Geoffrey Uy Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine

Search for other papers by Geoffrey Uy in
Current site
Google Scholar
PubMed
Close
 MD
,
Pankit Vachhani O’Neil Comprehensive Cancer Center at UAB

Search for other papers by Pankit Vachhani in
Current site
Google Scholar
PubMed
Close
 MD
,
Carly J. Cassara National Comprehensive Cancer Network

Search for other papers by Carly J. Cassara in
Current site
Google Scholar
PubMed
Close
 MSc
,
Deborah A. Freedman-Cass National Comprehensive Cancer Network

Search for other papers by Deborah A. Freedman-Cass in
Current site
Google Scholar
PubMed
Close
 PhD
, and
Katie Stehman National Comprehensive Cancer Network

Search for other papers by Katie Stehman in
Current site
Google Scholar
PubMed
Close
 PA-C, MMS
Restricted access

Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy characterized by the clonal expansion of myeloid blasts in the peripheral blood, bone marrow, and/or other tissues. It is the most common form of acute leukemia among adults and accounts for the largest number of annual deaths from leukemias in the United States. Like AML, blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a myeloid malignancy. It is a rare malignancy characterized by the aggressive proliferation of precursors of plasmacytoid dendritic cells that frequently involves the bone marrow, skin, central nervous system, and other organs and tissues. This discussion section focuses on the diagnosis and management of BPDCN as outlined in the NCCN Guidelines for AML.

Individual Disclosures for the Acute Myeloid Leukemia Panel
Individual Disclosures for the Acute Myeloid Leukemia Panel

  • Collapse
  • Expand
  • 1.

    Bueno C, Almeida J, Lucio P, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica 2004;89:5869.

  • 2.

    Guru Murthy GS, Pemmaraju N, Atallah E. Epidemiology and survival of blastic plasmacytoid dendritic cell neoplasm. Leuk Res 2018;73:2123.

  • 3.

    Aoki T, Suzuki R, Kuwatsuka Y, et al. Long-term survival following autologous and allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm. Blood 2015;125:35593562.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4.

    Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program 2016;2016:1623.

  • 5.

    Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:23912405.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6.

    Khoury JD, Solary E, Abla O, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia 2022;36:17031719.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Facchetti F, Petrella T, Pileri S. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, Campo E, Harris NL, et al., eds WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th ed. IARC; 2017:173177.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8.

    Pagano L, Valentini CG, Pulsoni A, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 2013;98:239246.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9.

    Khanlari M, Yin CC, Takahashi K, et al. Bone marrow clonal hematopoiesis is highly prevalent in blastic plasmacytoid dendritic cell neoplasm and frequently sharing a clonal origin in elderly patients. Leukemia 2022;36:13431350.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10.

    Dalle S, Beylot-Barry M, Bagot M, et al. Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol 2010;162:7479.

  • 11.

    Pemmaraju N, Lane AA, Sweet KL, et al. Tagraxofusp in blastic plasmacytoid dendritic-cell neoplasm. N Engl J Med 2019;380:16281637.

  • 12.

    Martín-Martín L, Almeida J, Pomares H, et al. Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. Oncotarget 2016;7:1017410181.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13.

    Feuillard J, Jacob MC, Valensi F, et al. Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood 2002;99:15561563.

  • 14.

    Tsagarakis NJ, Kentrou NA, Papadimitriou KA, et al. Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the Hellenic Dendritic Cell Leukemia Study Group. Leuk Res 2010;34:438446.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15.

    Sukswai N, Aung PP, Yin CC, et al. Dual expression of TCF4 and CD123 is highly sensitive and specific for blastic plasmacytoid dendritic cell neoplasm. Am J Surg Pathol 2019;43:14291437.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16.

    Ceribelli M, Hou ZE, Kelly PN, et al. A druggable TCF4- and BRD4- dependent transcriptional network sustains malignancy in blastic plasmacytoid dendritic cell neoplasm. Cancer Cell 2016;30:764778.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17.

    Wilson NR, Bover L, Konopleva M, et al. CD303 (BDCA-2) - a potential novel target for therapy in hematologic malignancies. Leuk Lymphoma 2022;63:1930.

  • 18.

    Menezes J, Acquadro F, Wiseman M, et al. Exome sequencing reveals novel and recurrent mutations with clinical impact in blastic plasmacytoid dendritic cell neoplasm. Leukemia 2014;28:823829.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Batta K, Bossenbroek HM, Pemmaraju N, et al. Divergent clonal evolution of blastic plasmacytoid dendritic cell neoplasm and chronic myelomonocytic leukemia from a shared TET2-mutated origin. Leukemia 2021;35:32993303.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Reimer P, Rüdiger T, Kraemer D, et al. What is CD4+CD56+ malignancy and how should it be treated? Bone Marrow Transplant 2003;32:637646.

  • 21.

    Frankel AE, Woo JH, Ahn C, et al. Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood 2014;124:385392.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22.

    Pemmaraju N, Sweet KL, Stein AS, et al. Long-term benefits of tagraxofusp for patients with blastic plasmacytoid dendritic cell neoplasm. J Clin Oncol 2022;40:30323036.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23.

    Deotare U, Yee KW, Le LW, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-color flow cytometry diagnosis and HyperCVAD therapy. Am J Hematol 2016;91:283286.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24.

    Pemmaraju N, Wilson NR, Garcia-Manero G, et al. Characteristics and outcomes of patients with blastic plasmacytoid dendritic cell neoplasm treated with frontline HCVAD. Blood Adv 2022;6:30273035.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25.

    DiNardo CD, Rausch CR, Benton C, et al. Clinical experience with the BCL2-inhibitor venetoclax in combination therapy for relapsed and refractory acute myeloid leukemia and related myeloid malignancies. Am J Hematol 2018;93:401407.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26.

    Prescribing information for venetoclax tablets, for oral use. 2022. Accessed March 24, 2023. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2022/208573s027lbl.pdf

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27.

    Montero J, Stephansky J, Cai T, et al. Blastic blasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax. Cancer Discov 2017;7:156164.

  • 28.

    Roos-Weil D, Dietrich S, Boumendil A, et al. Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation. Blood 2013;121:440446.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29.

    Kharfan-Dabaja MA, Al Malki MM, Deotare U, et al. Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study. Br J Haematol 2017;179:781789.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30.

    Bruch PM, Dietrich S, Finel H, et al. Retrospective analysis of hematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: conditioning intensity matters. Leukemia 2023;37:465472.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 31.

    Pemmaraju N, Kantarjian H, Sweet K, et al. North American Blastic Plasmacytoid Dendritic Cell Neoplasm Consortium: position on standards of care and areas of need. Blood 2023;141:567578.

    • PubMed
    • Search Google Scholar
    • Export Citation
All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 19227 15965 930
PDF Downloads 17805 15312 969
EPUB Downloads 0 0 0