Plasmacytoid dendritic cells in the setting of myeloid neoplasms: Diagnostic guide to challenging pathologic presentations

Hussein, Siba El; Wang, Wei

doi:10.1111/bjh.18632

Cited by 10 publications

(17 citation statements)

References 56 publications

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“…myelodysplastic neoplasm (MDS) [9,17] and myeloproliferative neoplasm (MPN) [16,17], pDCs show a spectrum of maturation ranging from early pDCs to fully mature pDCs and are believed to originate from early pDC precursors [8].…”

Section: Bpdcnmentioning

confidence: 99%

Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms

El Hussein,

Wang

2024

Cancers

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In this review, we aim to provide a summary of the diverse immunophenotypic presentations of distinct entities associated with plasmacytoid dendritic cell (pDC) proliferation. These entities include the following: (1) blastic plasmacytoid dendritic cell neoplasm (BPDCN); (2) mature pDC proliferation (MPDCP), most commonly seen in chronic myelomonocytic leukemia (CMML); and (3) myeloid neoplasms with pDC differentiation, in which pDCs show a spectrum of maturation from early immature pDCs to mature forms, most commonly seen in acute myeloid leukemia (pDC-AML). Our aim is to provide a flow cytometry diagnostic approach to these distinct and sometimes challenging entities and to clarify the immunophenotypic spectrum of neoplastic pDCs in different disease presentations. In this review, we also cover the strategies in the evaluation of residual disease, as well as the challenges and pitfalls we face in the setting of immune and targeted therapy. The differential diagnosis will also be discussed, as blasts in some AML cases can have a pDC-like immunophenotype, mimicking pDCs.

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Section: Bpdcnmentioning

confidence: 99%

Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms

El Hussein,

Wang

2024

Cancers

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“…Another scenario is the evolution of MN to blastic plasmacytoid dendritic cell neoplasm (BPDCN) [120,121]. Such rare progressions have been observed in the context of MDS, CMML, and MPN [122][123][124].…”

Section: Progression In Myeloid Neoplasmsmentioning

confidence: 99%

“…In many cases, both compounds share a common clonal origin. This can be partially explained by the high prevalence of clonal hematopoiesis in elderly patients [125], with a common precursor cell giving rise to MN, which subsequently transforms and gives rise to BPDCN (linear evolution) or, more likely, a common precursor cell giving separate rise to the BPDCN and the MN (branched evolution) [120]. The most commonly documented mutations upon progression are TET2 , ASXL1 and ZRSR2TET2 , ASXL1 and ZRSR2 [125, 126].…”

Section: Histiocytic/dendritic Outgrowthsmentioning

confidence: 99%

Progression in Myeloid Neoplasms: Beyond the Myeloblast

Faria

Tzankov

2023

Pathobiology

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Disease progression in myelodysplastic syndromes (MDS), myelodysplastic-myeloproliferative neoplasms (MDS/MPN), and myeloproliferative neoplasms (MPN), altogether referred to as myeloid neoplasms (MN), is a major source of mortality. Apart from transformation to acute myeloid leukemia, the clinical progression of MN is mostly due to the overgrowth of pre-existing hematopoiesis by the MN without an additional transforming event. Still, MN may evolve along other recurrent yet less well-known scenarios: (1) acquisition of MPN features in MDS or (2) MDS features in MPN, (3) progressive myelofibrosis (MF), (4) acquisition of chronic myelomonocytic leukemia (CMML)-like characteristics in MPN or MDS, (5) development of myeloid sarcoma (MS), (6) lymphoblastic (LB) transformation, (7) histiocytic/dendritic outgrowths. These MN-transformation types exhibit a propensity for extramedullary sites (e.g., skin, lymph nodes, liver), highlighting the importance of lesional biopsies in diagnosis. Gain of distinct mutations/mutational patterns seems to be causative or at least accompanying several of the above-mentioned scenarios. MDS developing MPN features often acquire MPN driver mutations (usually JAK2), and MF. Conversely, MPN gaining MDS features develop, e.g., ASXL1, IDH1/2, SF3B1, and/or SRSF2 mutations. Mutations of RAS-genes are often detected in CMML-like MPN progression. MS ex MN is characterized by complex karyotypes, FLT3 and/or NPM1 mutations, and often monoblastic phenotype. MN with LB transformation is associated with secondary genetic events linked to lineage reprogramming leading to the deregulation of ETV6, IKZF1, PAX5, PU.1, and RUNX1. Finally, the acquisition of MAPK-pathway gene mutations may shape MN toward histiocytic differentiation. Awareness of all these less well-known MN-progression types is important to guide optimal individual patient management.

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“…MPDCP associated with myeloid neoplasm manifests as nodules or aggregates of mature pDCs in the lymph nodes, skin, and bone marrow [ 4 , 5 ]. MPDCP represents a morphologically mature, low-grade proliferation of pDCs, characterized by the expression of pDC markers (including CD123, CD303, CD304, TCF4), a low proliferation index (<10% Ki-67), and the absence or low expression of CD56 [ 6 ].…”

Section: Mature Plasmacytoid Dendritic Cell Proliferation (Mpdcp) Ass...mentioning

confidence: 99%

“…This is based on CD56 positivity and the presence of pDC markers ( Fig. 1 ) [ 5 ]. Compared to BPDCN, pDC-AML exhibits RUNX1 mutations, fewer skin lesions, and expresses CD34-positive and CD56-negative pDCs [ 8 ].…”

Section: Blastic Plasmacytoid Dendritic Cell Neoplasmmentioning

confidence: 99%

Plasmacytoid dendritic cell neoplasms

et al. 2023

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Plasmacytoid dendritic cells (pDCs) are type I interferon-producing cells that modulate immune responses. There are two types of pDC neoplasms: 1) mature pDC proliferation (MPDCP) associated with myeloid neoplasm and 2) blastic pDC neoplasm (BPDCN). MPDCP is a clonal expansion of mature pDCs that is predominantly associated with chronic myelomonocytic leukemia. In contrast, BPDCN is a clinically aggressive myeloid malignancy involving the skin, bone marrow, lymphatic organs, and central nervous system. There are various types of skin lesions, ranging from solitary brown or violaceous to disseminated cutaneous lesions, which often spread throughout the body. The expression of CD4, CD56, CD123, and pDC markers (TCL-1, TCF4, CD303, and CD304, etc.) are typical immunophenotype of BPDCN. Historically, BPDCN treatment has been based on acute leukemia regimens and allogeneic hematopoietic cell transplantation in selected patients. Recent advances in molecular biology and genetics have led to the development of targeted agents, such as tagraxofusp (a recombinant fusion protein targeting CD123), anti-CD123 CAR-T cells, XmAb14045, and IMGN632. Lastly, this review provides a comprehensive overview of pDC neoplasms.

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Plasmacytoid dendritic cells in the setting of myeloid neoplasms: Diagnostic guide to challenging pathologic presentations

Cited by 10 publications

References 56 publications

Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms

Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms

Progression in Myeloid Neoplasms: Beyond the Myeloblast

Plasmacytoid dendritic cell neoplasms

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