Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-77c89778f8-gvh9x Total loading time: 0 Render date: 2024-07-17T17:44:23.235Z Has data issue: false hasContentIssue false

Chapter 16 - Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs)

from Section IV - Neoplastic Disorders of Bone Marrow

Published online by Cambridge University Press:  25 January 2024

By
Bradford Siegele
Edited by
Xiayuan Liang ,
Bradford Siegele  and
Jennifer Picarsic
Xiayuan Liang
Affiliation:
Children’s Hospital of Colorado
Bradford Siegele
Affiliation:
Children’s Hospital of Colorado
Jennifer Picarsic
Affiliation:
Cincinnati Childrens Hospital Medicine Center
Get access

Summary

Blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are an aggressive hematopoietic malignancy derived from precursors to plasmacytoid dendritic neoplasms, with a predilection toward involvement of the skin, bone marrow, and peripheral blood.1

Type
Chapter
Information
Pediatric Pathology of Hematopoietic and Histiocytic Disorders , pp. 196 - 198
Publisher: Cambridge University Press
Print publication year: 2024

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Swerdlow, S, Campo, E, Harris, N, et al., eds. WHO classification of tumors of haematopoietic and lymphoid tissues. Rev. 4th ed. IARC Press; 2017.Google Scholar
Deng, W, Yang, M, Kuang, F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: a review of two cases. Mol Clin Oncol. 2017;7(4):709–15. doi: 10.3892/mco.2017.1370Google Scholar
Li, Y, Sun, V, Sun, W, et al. Blastic plasmacytoid dendritic cell neoplasm in children. Hematol Oncol Clin North Am. 2020;34(3):601–12. doi: 10.1016/j.hoc.2020.01.008Google Scholar
Jegalian, AG, Buxbaum, NP, Facchetti, F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: diagnostic features and clinical implications. Haematologica. 2010;95(11):1873–9. doi: 10.3324/haematol.2010.026179Google Scholar
Feuillard, J, Jacob, MC, Valensi, F, et al. Clinical and biologic features of CD4(+) CD56(+) malignancies. Blood. 2002;99(5):1556–63. doi:10.1182/blood.v99.5.1556Google Scholar
Jacob, MC, Chaperot, L, Mossuz, P, et al. CD4+ CD56+ lineage negative malignancies: a new entity developed from malignant early plasmacytoid dendritic cells. Haematologica. 2003;88(8):941–55.Google Scholar
Petrella, T, Bagot, M, Willemze, R, et al. Blastic NK-cell lymphomas (agranular CD4+ CD56+ hematodermic neoplasms): a review. Am J Clin Pathol. 2005;123(5):662–75.Google Scholar
Cota, C, Vale, E, Viana, I, et al. Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients.Am J Surg Pathol. 2010;34(1):75–87. doi:10.1097/PAS.0b013e3181c5e26bGoogle Scholar
Pilichowska, ME, Fleming, MD, Pinkus, JL, et al. CD4+/CD56+ hematodermic neoplasm (“blastic natural killer cell lymphoma”): neoplastic cells express the immature dendritic cell marker BDCA-2 and produce interferon. Am J Clin Pathol. 2007;128(3):445–53. doi: 10.1309/W9Q5AGYDE5LANN39Google Scholar
Urosevic, M, Conrad, C, Kamarashev, J, et al. CD4+ CD56+ hematodermic neoplasms bear a plasmacytoid dendritic cell phenotype. Hum Pathol. 2005;36(9):1020–4. doi: 10.1016/j.humpath.2005.07.002Google Scholar
Leroux, D, Mugneret, F, Callanan, M, et al. CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique. Blood. 2002;99(11):4154–9. doi:10.1182/blood.v99.11.4154Google Scholar
Sakamoto, K, Katayama, R, Asaka, R, et al. Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response. Leukemia. 2018;32(12):2590–603. doi:10.1038/s41375-018-0154-5Google Scholar
Tang, Z, Li, Y, Wang, W, et al. Genomic aberrations involving 12p/ETV6 are highly prevalent in blastic plasmacytoid dendritic cell neoplasms and might represent early clonal events. Leuk Res. 2018;73:86–94. doi: 10.1038/s41375-018-0154-5Google Scholar
Dijkman, R, van Doorn, R, Szuhai, K, et al. Gene-expression profiling and array-based CGH classify CD4+CD56+ hematodermic neoplasm and cutaneous myelomonocytic leukemia as distinct disease entities. Blood. 2007;109(4):1720–7. doi:10.1182/blood-2006-04-018143CrossRefGoogle ScholarPubMed
Sapienza, MR, Fuligni, F, Agostinelli, C, et al. Molecular profiling of blastic plasmacytoid dendritic cell neoplasm reveals a unique pattern and suggests selective sensitivity to NF-kB pathway inhibition. Leukemia. 2014;28(8):1606–16. doi: 10.1038/leu.2014.64Google Scholar
Alayed, K, Patel, KP, Konoplev, S, et al. TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow. Am J Hematol. 2013;88(12):1055–61. doi:10.1002/ajh.23567Google Scholar
Suzuki, K, Suzuki, Y, Hama, A, et al. Recurrent MYB rearrangement in blastic plasmacytoid dendritic cell neoplasm. Leukemia. 2017;31(7):1629–33. doi: 10.1038/leu.2017.101Google Scholar
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(5):764–78. doi:10.1016/j.ccell.2016.10.002Google Scholar
Jegalian, AG, Facchetti, F, Jaffe, ES. Plasmacytoid dendritic cells: physiologic roles and pathologic states. Adv Anat Pathol. 2009;16(6):392–404. doi: 10.1097/PAP.0b013e3181bb6bc2Google Scholar
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(3):440–6. doi: 10.1182/blood-2012-08-448613Google Scholar
Reimer, P, Rüdiger, T, Kraemer, D, et al. What is CD4+ CD56+ malignancy and how should it be treated? Bone Marrow Transplant. 2003;32(7):637–46. doi: 10.1038/sj.bmt.1704215Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×