Dendritic Cell Leukemia: a Review

Tsagarakis, Nikolaos J.; Paterakis, Georgios

doi:10.1007/s11912-020-00921-y

Cited by 10 publications

(19 citation statements)

References 128 publications

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“…Dear Editor, Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematological malignancy arising from the precursors of plasmacytoid dendritic cells (pDC) with a high frequency of primary cutaneous involvement, and dissemination to bone marrow with disease progression. [1][2] In 2016 the World Health Organization (WHO) classification update of the acute myeloid leukemia (AML), the disease has been given its separate category under myeloid neoplasms. [3][4] Cutaneous involvement is often the first manifestation of the BPDCN, with subsequent or simultaneous spread to bone marrow.…”

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confidence: 99%

“…Leukemia as the first presenting symptom is a sporadic finding and can masquerade as acute undifferentiated leukemia. [1][2][5][6] BPDCN overall incidence is extremely low, accounting for 0.44% of all hematologic malignancies and 0.7% of cutaneous lymphomas. 1 BPDCN affects males predominantly (male/female ratio of 3.3), with 61 years as the median age of diagnosis.…”

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confidence: 99%

“…[1][2][5][6] BPDCN overall incidence is extremely low, accounting for 0.44% of all hematologic malignancies and 0.7% of cutaneous lymphomas. 1 BPDCN affects males predominantly (male/female ratio of 3.3), with 61 years as the median age of diagnosis. [1][2][7][8][9] It is exceedingly rare to see BPDCN with a leukemic presentation (BPDCL, Blastic Plasmacytoid Dendritic Cell Leukemia) at the onset, and the leukemic form of the disease are reported in 1% of cases of acute leukemia.…”

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confidence: 99%

“…1 BPDCN affects males predominantly (male/female ratio of 3.3), with 61 years as the median age of diagnosis. [1][2][7][8][9] It is exceedingly rare to see BPDCN with a leukemic presentation (BPDCL, Blastic Plasmacytoid Dendritic Cell Leukemia) at the onset, and the leukemic form of the disease are reported in 1% of cases of acute leukemia. [5][6] With the improvement in the understanding of the disease biology, it has become crucial to extend the epidemiology of this life-threatening hematologic malignancy.…”

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Blastic plasmocitoid dendritic cell neoplasm with leukemic spread: a GIMEMA survey

et al. 2021

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Blastic plasmocitoid dendritic cell neoplasm with leukemic spread: a GIMEMA survey

et al. 2021

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“…Ключевые слова: лейкоз из плазмоцитоидных дендритных клеток, иммунофенотипирование Л ейкоз из плазмоцитоидных дендритных клеток (ПДКЛ) является редким, клинически агрессивным новообразованием, происходящим из плазмоцитоидных дендритных клеток-предшественников [ Иммунофенотипические критерии диагностики ПДКЛ по N.J. Tsagarakis и соавт. [15] Figure 1 Immunophenotyping criteria for BPDCN diagnosis according to N.J. Tsagarakis et al [15] Обязательные критерии Indispensable criteria Критерии 1-й линии (не менее 6 из 8) First-line criteria (at least 6 out of 7) Критерии 2-й линии (не менее 5 из 7) Second-line criteria (at least 5 out 8)…”

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The diagnostics of blastic plasmocytoid dendritic cell neoplasm: report of five cases

Дёмина

Kashpor

Illarionova

et al. 2021

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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The diagnosis of rare hematological disorders requires a comprehensive clinical and laboratory investigation with careful interpretation of all test results. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is one of such rare entities. We have performed a retrospective analysis of the results of immunophenotyping, cytomorphology and cytogenetics of bone marrow tumor cells from 5 patients with BPDCN aged from 8 to 51 years. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. No specific characteristics of blasts were found. No correlation with the treatment and outcomes was noted as well: 3 patients died of progression or relapse (2 and 1, respectively). Bone marrow immunophenotyping is probably the most valuable laboratory test which allows physicians to establish the proper diagnosis in the absence of skin lesions. Flow cytometry immunophenotyping is the only technique used to determine the antigen profile that enables us to distinguish normal plasmacytoid dendritic cells from tumor ones by the presence (or absence) of the expression of CD2, CD7, CD38, CD56, CD303 etc. In the present paper, we provide a detailed description of five cases of BPDCN and main methods for flow cytometry data analysis. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.

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Diagnostic management of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in close interaction with therapeutic considerations

Shumilov,

Mazzeo,

Ghandili

et al. 2024

Ann Hematol

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare malignancy derived from plasmacytoid dendritic cells, can mimic both acute leukemia and aggressive T-cell lymphoma. Therapy of this highly aggressive hematological disease should be initiated as soon as possible, especially in light of novel targeted therapies that have become available. However, differential diagnosis of BPDCN remains challenging. This retrospective study aimed to highlight the challenges to timely diagnoses of BPDCN. We documented the diagnostic and clinical features of 43 BPDCN patients diagnosed at five academic hospitals from 2001–2022. The frequency of BPDCN diagnosis compared to AML was 1:197 cases. The median interval from the first documented clinical manifestation to diagnosis of BPDCN was 3 months. Skin (65%) followed by bone marrow (51%) and blood (45%) involvement represented the most common sites. Immunophenotyping revealed CD4 + , CD45 + , CD56 + , CD123 + , HLA-DR + , and TCL-1 + as the most common surface markers. Overall, 86% (e.g. CD33) and 83% (e.g., CD7) showed co-expression of myeloid and T-cell markers, respectively. In the median, we detected five genomic alterations per case including mutational subtypes typically involved in AML: DNA methylation (70%), signal transduction (46%), splicing factors (38%), chromatin modification (32%), transcription factors (32%), and RAS pathway (30%), respectively. The contribution of patients (30%) proceeding to any form of upfront stem cell transplantation (SCT; autologous or allogeneic) was almost equal resulting in beneficial overall survival rates in those undergoing allogeneic SCT (p = 0.0001). BPDCN is a rare and challenging entity sharing various typical characteristics of other hematological diseases. Comprehensive diagnostics should be initiated timely to ensure appropriate treatment strategies.

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Dendritic Cell Leukemia: a Review

Cited by 10 publications

References 128 publications

Blastic plasmocitoid dendritic cell neoplasm with leukemic spread: a GIMEMA survey

Blastic plasmocitoid dendritic cell neoplasm with leukemic spread: a GIMEMA survey

The diagnostics of blastic plasmocytoid dendritic cell neoplasm: report of five cases

Diagnostic management of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in close interaction with therapeutic considerations

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