Blastic Plasmacytoid Dendritic Cell Neoplasm–Current Insights

Venugopal, Sangeetha; Zhou, Selena; Jamal, Siraj M. El; Lane, Andrew A.; Mascarenhas, John

doi:10.1016/j.clml.2019.06.002

Cited by 45 publications

(66 citation statements)

References 85 publications

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“…Blastic plasmacytoid dendritic cell neoplasm (BPDCN) represents the malignancy with the most promising clinical applications of CD123 targeted therapy. BPDCN is an extremely rare clonal hematological malignancy of plasmacytoid dendritic cell precursors, usually affecting elderly males and presenting in the skin with frequent involvement of the bone marrow, peripheral blood and lymph nodes [73,74]. The epidemiology, pathology, molecular abnormalities and clinical features of BPDCN were recently reviewed [73,74].…”

Section: Introductionmentioning

confidence: 99%

CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies

Testa

Pelosi

Castelli

2019

Cancers

108

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The interleukin-3 receptor alpha chain (IL-3Rα), more commonly referred to as CD123, is widely overexpressed in various hematological malignancies, including acute myeloid leukemia (AML), B-cell acute lymphoblastic leukemia, hairy cell leukemia, Hodgkin lymphoma and particularly, blastic plasmacytoid dendritic neoplasm (BPDCN). Importantly, CD123 is expressed at both the level of leukemic stem cells (LSCs) and more differentiated leukemic blasts, which makes CD123 an attractive therapeutic target. Various agents have been developed as drugs able to target CD123 on malignant leukemic cells and on the normal counterpart. Tagraxofusp (SL401, Stemline Therapeutics), a recombinant protein composed of a truncated diphtheria toxin payload fused to IL-3, was approved for use in patients with BPDCN in December of 2018 and showed some clinical activity in AML. Different monoclonal antibodies directed against CD123 are under evaluation as antileukemic drugs, showing promising results either for the treatment of AML minimal residual disease or of relapsing/refractory AML or BPDCN. Finally, recent studies are exploring T cell expressing CD123 chimeric antigen receptor-modified T-cells (CAR T) as a new immunotherapy for the treatment of refractory/relapsing AML and BPDCN. In December of 2018, MB-102 CD123 CAR T developed by Mustang Bio Inc. received the Orphan Drug Designation for the treatment of BPDCN. In conclusion, these recent studies strongly support CD123 as an important therapeutic target for the treatment of BPDCN, while a possible in the treatment of AML and other hematological malignancies will have to be evaluated by in the ongoing clinical studies.

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Section: Introductionmentioning

confidence: 99%

CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies

Testa

Pelosi

Castelli

2019

Cancers

108

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“…Recently, the presence of four out of five characteristic markers of BPDCN, namely, CD4, CD56, CD123, CD303, and TCL1, has been shown to aid in the accurate diagnosis of BPDCN 2 . Skin lesions commonly occur in a majority of patients with BPDCN, and blasts of BPDCN are suggested to arise from premalignant hematopoietic precursor clones 3 . Thus, patients with systemic lesions were treated with conventional chemotherapies for acute lymphoblastic leukemia, acute myeloid leukemia, or non‐Hodgkin’s lymphoma, sometimes followed by autologous or allogeneic hematopoietic cell transplantation as a consolidation therapy 4 .…”

Section: Introductionmentioning

confidence: 99%

Severe tumor lysis syndrome during the induction therapy for the treatment of blastic plasmacytoid dendritic cell neoplasm arising from myelodysplastic/myeloproliferative neoplasms

Sagou

Itō

Kawamura

et al. 2020

Clinical Case Reports

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“…BPDCN is an aggressive malignancy originating from plasmacytoid dendritic cell (pDC) precursors. 1 The term BPDCN was established in the 2008 World Health Organization classification of lymphomas and included in the group of acute myeloid leukemias (AML) and related neoplasms, but was reclassified as a distinct entity in 2016. 2 It is a rare disease (0.7% of all primary cutaneous lymphomas), with an incidence of 0.04 cases/ 100 000.…”

Section: Discussionmentioning

confidence: 99%

“…2 It is a rare disease (0.7% of all primary cutaneous lymphomas), with an incidence of 0.04 cases/ 100 000. 1 It predominantly affects elderly males and it is extremely rare in children. 1 In one of the largest reviews, Kim The disease has a marked predilection for cutaneous involvement.…”

Section: Discussionmentioning

confidence: 99%

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Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management

Rivas-Calderón¹,

Cheirif-Wolosky²,

Rosas-Romero

et al. 2020

Pediatric Dermatology

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A 13-year-old boy was referred with a 10-month history of an asymptomatic dark violaceous infiltrated nodular lesion on the left cheek (Figure 1). He had been previously treated with topical and systemic steroids with partial improvement. A skin biopsy revealed a dense dermal infiltrate of monomorphous, poorly differentiated medium-sized cells with blastic morphology (Figure 2A-B). Immunohistochemical staining revealed positive CD4, CD56, and CD123 cells that were negative for CD3, CD8, CD20, CD10, PAX5, TdT, LMP1, and MPO; Ki67 index was 40% (Figure 2C). The diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was made. Bone marrow (BM) biopsy and cerebrospinal fluid (CSF) examination were normal. Positron emission tomography-computed tomography (PET/CT) revealed hypermetabolic activity (SUVmax 4.68) localized to the skin. After careful risk-benefit deliberation with a multidisciplinary team, systemic treatment with a high-risk acute lymphoblastic leukemia (ALL) chemotherapy protocol was initiated (vincristine, dexamethasone, asparaginase, daunomycin, cytarabine, cyclophosphamide, and methotrexate). The patient is currently receiving treatment and has had good response without complications after 18 months' follow-up. How to cite this article: Rivas-Calderón MK, Cheirif-Wolosky O, Rosas-Romero ME, et al. Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management.

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Blastic Plasmacytoid Dendritic Cell Neoplasm–Current Insights

Cited by 45 publications

References 85 publications

CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies

CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies

Severe tumor lysis syndrome during the induction therapy for the treatment of blastic plasmacytoid dendritic cell neoplasm arising from myelodysplastic/myeloproliferative neoplasms

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management

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