Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects

Sapienza, Maria Rosaria; Pileri, Alessandro; Derenzini, Enrico; Melle, Federica; Motta, Giovanna; Fiori, Stefano; Calleri, Angelica; Pimpinelli, Nicola; Tabanelli, Valentina; Pileri, Stefano

doi:10.3390/cancers11050595

Cited by 78 publications

(95 citation statements)

References 114 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Regimens for AML/ALL like hyper-CVAD, methotrexate and cytarabine, lymphoma chemotherapies like CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) and CHEOP (CHOP plus etoposide) may be effective 1 7 8 15 16. CNS prophylaxis using intrathecal therapy is highly recommended for BPDCN given the high risk of CNS involvement especially in relapses 1 7 8 15 16. Newer modalities include agents like venetoclax which is a BCL2 inhibitor effective in BPDCN 7 17.…”

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia

et al. 2019

View full text Add to dashboard Cite

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare haematological malignancy defined by concurrent expression of CD4, CD56, BCL-2 and CD123. The disease has a very poor prognosis and there are no well-established treatment guidelines. We describe a case of BPDCN in a 65-year-old female patient with myeloproliferative disorder (essential thrombocythemia) and chronic lymphocytic leukaemia. She presented with rapidly progressive facial and scalp lesions. Skin biopsy confirmed BPDCN and the imaging revealed widespread disease. Patient was started on hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) and intrathecal methotrexate. Due to progression on initial treatment, she was treated with decitabine and venetoclax (BCL-2 inhibitor). However, patient continued to deteriorate and died after 4 months from initial diagnosis. We emphasise on the clinical features, emerging treatment modalities and prognosis of BPDCN.

show abstract

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Multiagent chemotherapy regimens as in ALL are the most accepted applications for these patients. The disease often relapses after chemotherapy and becomes resistant to the previous drugs [3,4,11,13,23,25].…”

Section: Antibodymentioning

confidence: 99%

“…The tumor cells typically express CD4, CD56, CD43, CD45RA, and plasmacytoid dendritic cell antigens (CD123, CD303, TCL1A, CD2AP, SPIB, and type 1 interferon-dependent molecule MX1) [3]. The molecular profile showed that this entity is much more related to myeloid neoplasms [4].…”

Section: Introductionmentioning

confidence: 99%

Blastic Plasmacytoid Dendritic Cell Neoplasia: A Single Center Experience

Demiröz

Demirkesen

Salihoğlu

et al. 2019

Tjh

View full text Add to dashboard Cite

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignancy with skin tropism. The entity was recently defined and the diagnosis is generally made by skin biopsies. It is necessary to apply appropriate immunohistochemistry to recognize this rare entity. There is no consensus on therapy and the survival rates are low. The aim of this study is to describe the clinical and histopathological features of BPDCN. We retrospectively reviewed 8 BPDCN cases of the Cerrahpaşa Medical Faculty diagnosed between 2005 and 2019. We documented the clinical findings, histopathologic diagnoses, and outcomes. The mean age of the patients was 58.7 years (range=11-86 years), and 7 patients were male. The patients presented with erythematous or purple papules, plaques, and papulonodular or nodular cutaneous lesions. Two had lymphadenomegaly at presentation. In microscopic evaluations, tumor cells infiltrated the entire dermis with a clear-cut subepidermal Grenz zone in all cases. CD4, CD56, and CD123 were the most frequently expressed immunohistochemical markers. The median follow-up of 7 cases was 14 months, ranging from 6 to 48 months. Three patients died of the disease, while 4 patients were still alive. Out of 7 patients, 5 received chemotherapy. We found that the outcomes of some patients were different from others but we did not link any distinct clinical or histopathological characteristics to these different outcomes.

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects

Cited by 78 publications

References 114 publications

Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia

Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia

Blastic Plasmacytoid Dendritic Cell Neoplasia: A Single Center Experience

CD123 as a Therapeutic Target in the Treatment of Hematological Malignancies

Contact Info

Product

Resources

About