Early T-cell Precursor Lymphoblastic Leukemia Accompanied by Prominent Blastic Plasmacytoid Dendritic Cell Proliferation Mimicking Blastic Plasmacytoid Dendritic Cell Neoplasm: an Exceptional Case Report and Literature Review

Overview

Journal J Cancer Res Clin Oncol

Specialty Oncology

Date 2022 Aug 6

PMID 35933443

Authors

Hongyan Liao

Jiang Yu

Yu Liu

Sha Zhao

Huanling Zhu

Dongsheng Xu

Nenggang Jiang

Qin Zheng

Affiliations

Soon will be listed here.

Abstract

Purpose: Plasmacytoid dendritic cells (pDCs) are commonly associated with myeloid malignancies. The association between lymphoblastic leukemia and pDCs has been little explored.

Case Presentation: Here, we report a novel case of early T-cell precursor lymphoblastic leukemia (ETP-ALL) accompanied by prominent proliferation of blastic pDCs mimicking BPDCN. The diagnosis was established based on a comprehensive analysis of morphology, immunophenotype and clinical implications. We also present a literature review and discussion on the differential expression of reactive and neoplastic pDCs, the functional role of pDCs in lymphoblastic leukemia, and the etiological association of normal pDCs and BPDCN.

Conclusions: The current case demonstrates for the first time that prominent pDC proliferation can be associated with lymphoid neoplasms and can exhibit blastic morphology and immunophenotype. The underlying mechanism of the coexistence of these two blastic populations remains unknown. Further genetic profiling may be required to denote the progressive development of tumor stem cells to the lymphoid, myeloid or dendritic cell lineage. Moreover, the prognostic value of pDCs in hematological neoplasms needs further investigation.

Citing Articles

Case report: single-cell transcriptome sequencing reveals the clonal origin of mature plasmacytoid dendritic cell proliferation in early T-cell precursor lymphoblastic leukemia.

Zhang L, Lu Y, Jiang J, Wu G Ann Hematol. 2025; .

PMID: 40085211 DOI: 10.1007/s00277-025-06301-5.

References

Alayed K, Patel K, Konoplev S, Singh R, Routbort M, Reddy N . 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.23567. View

Cordeau M, Belounis A, Lelaidier M, Cordeiro P, Sartelet H, Herblot S . Efficient Killing of High Risk Neuroblastoma Using Natural Killer Cells Activated by Plasmacytoid Dendritic Cells. PLoS One. 2016; 11(10):e0164401. PMC: 5055353. DOI: 10.1371/journal.pone.0164401. View

Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L . Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol. 2009; 34(1):75-87. DOI: 10.1097/PAS.0b013e3181c5e26b. View

Coustan-Smith E, Mullighan C, Onciu M, Behm F, Raimondi S, Pei D . Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia. Lancet Oncol. 2009; 10(2):147-56. PMC: 2840241. DOI: 10.1016/S1470-2045(08)70314-0. View

Dalle S, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P . Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?. Br J Dermatol. 2009; 162(1):74-9. DOI: 10.1111/j.1365-2133.2009.09373.x. View

Dargent J, Delannoy A, Pieron P, Husson B, Debecker C, Petrella T . Cutaneous accumulation of plasmacytoid dendritic cells associated with acute myeloid leukemia: a rare condition distinct from blastic plasmacytoid dendritic cell neoplasm. J Cutan Pathol. 2011; 38(11):893-8. DOI: 10.1111/j.1600-0560.2011.01777.x. View

Derolf A, Laane E, Bjorklund E, Saft L, Bjorkholm M, Porwit A . Dendritic cells in bone marrow at diagnosis and after chemotherapy in adult patients with acute myeloid leukaemia. Scand J Immunol. 2014; 80(6):424-31. DOI: 10.1111/sji.12223. View

Diaz-Rodriguez Y, Cordeiro P, Belounis A, Herblot S, Duval M . In vitro differentiated plasmacytoid dendritic cells as a tool to induce anti-leukemia activity of natural killer cells. Cancer Immunol Immunother. 2017; 66(10):1307-1320. PMC: 5626790. DOI: 10.1007/s00262-017-2022-y. View

Feuillard J, Jacob M, Valensi F, Maynadie M, Gressin R, Chaperot L . Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood. 2002; 99(5):1556-63. DOI: 10.1182/blood.v99.5.1556. View

10.

Frankel A, Woo J, Ahn C, Pemmaraju N, Medeiros B, Carraway H . Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood. 2014; 124(3):385-92. PMC: 4260361. DOI: 10.1182/blood-2014-04-566737. View

11.

Fu Y, Fesler M, Mahmud G, Bernreuter K, Jia D, Batanian J . Narrowing down the common deleted region of 5q to 6.0 Mb in blastic plasmacytoid dendritic cell neoplasms. Cancer Genet. 2013; 206(7-8):293-8. DOI: 10.1016/j.cancergen.2013.07.002. View

12.

Gabrilovich D . Mechanisms and functional significance of tumour-induced dendritic-cell defects. Nat Rev Immunol. 2004; 4(12):941-52. DOI: 10.1038/nri1498. View

13.

Grouard G, Rissoan M, Filgueira L, Durand I, Banchereau J, Liu Y . The enigmatic plasmacytoid T cells develop into dendritic cells with interleukin (IL)-3 and CD40-ligand. J Exp Med. 1997; 185(6):1101-11. PMC: 2196227. DOI: 10.1084/jem.185.6.1101. View

14.

Harvell J, Fulton R, Jones C, Terris D, Warnke R . Composite dendritic cell neoplasm (NOS) and small lymphocytic lymphoma. Appl Immunohistochem Mol Morphol. 2000; 8(4):322-8. View

15.

Herling M, Teitell M, Shen R, Medeiros L, Jones D . TCL1 expression in plasmacytoid dendritic cells (DC2s) and the related CD4+ CD56+ blastic tumors of skin. Blood. 2003; 101(12):5007-9. DOI: 10.1182/blood-2002-10-3297. View

16.

Ishikawa F, Niiro H, Iino T, Yoshida S, Saito N, Onohara S . The developmental program of human dendritic cells is operated independently of conventional myeloid and lymphoid pathways. Blood. 2007; 110(10):3591-660. PMC: 2077309. DOI: 10.1182/blood-2007-02-071613. View

17.

Jain N, Lamb A, OBrien S, Ravandi F, Konopleva M, Jabbour E . Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype. Blood. 2016; 127(15):1863-9. PMC: 4915808. DOI: 10.1182/blood-2015-08-661702. View

18.

Jegalian A, Facchetti F, Jaffe E . Plasmacytoid dendritic cells: physiologic roles and pathologic states. Adv Anat Pathol. 2009; 16(6):392-404. PMC: 6329307. DOI: 10.1097/PAP.0b013e3181bb6bc2. View

19.

Jing Y, Shaheen E, Drake R, Chen N, Gravenstein S, Deng Y . Aging is associated with a numerical and functional decline in plasmacytoid dendritic cells, whereas myeloid dendritic cells are relatively unaltered in human peripheral blood. Hum Immunol. 2009; 70(10):777-84. PMC: 5718338. DOI: 10.1016/j.humimm.2009.07.005. View

20.

Julia F, Dalle S, Duru G, Balme B, Vergier B, Ortonne N . Blastic plasmacytoid dendritic cell neoplasms: clinico-immunohistochemical correlations in a series of 91 patients. Am J Surg Pathol. 2014; 38(5):673-80. DOI: 10.1097/PAS.0000000000000156. View