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Blastic plasmacytoid dendritic cell neoplasm: Extensive disease, Langer lines, and blood burden in a retrospective study of 66 cases.
J Am Acad Dermatol
November 2024
Department of Dermatology, Brigham and Women's Hospital, and the Center for Cutaneous Oncology, Dana-Farber/Brigham Cancer Center, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts. Electronic address:
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Single-Center Experience. Clinical Characterization, Mutational Landscape, and Clinical Outcome of Patients Undergoing Hematopoietic Stem Cell Transplantation Intensive Therapy.
Actas Dermosifiliogr
November 2024
Department of Dermatology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, España. Electronic address:
Article Synopsis
- - Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive type of skin cancer, often presenting as violaceous nodules or bruise-like plaques, with most cases showing significant skin involvement.
- - The cancer cells typically express specific markers (CD4, CD56, CD123, CD303) and common genetic mutations include TET2 and NRAS, identified through advanced genetic sequencing techniques.
- - Patients treated with aggressive chemotherapy followed by allogenic stem cell transplantation had better outcomes, especially younger patients or those receiving treatments similar to those for acute lymphoblastic leukemia (ALL).
Blastic plasmacytoid dendritic cell neoplasm or CD4+ CD56+ acute myeloid leukemia: A case report.
Ann Biol Clin (Paris)
September 2024
Hematology Laboratory, Aziza Othmana Hospital Tunis, , Kasbah Street, Tunis, Tunisia.
Article Synopsis
- Blastic plasmacytoid dendritic-cell neoplasm (BPDCN) is a rare and aggressive cancer that arises from dendritic cells and is known for its poor prognosis and unique surface markers (CD4 and CD56).
- The disease has had various names over time, highlighting the complications in understanding its origin and diagnosing it, particularly due to similarities with other conditions like acute myeloid leukemia (AML) and histiocytic sarcoma (HS).
- A case study of a 42-year-old male revealed diagnostic challenges, as initial suspicions of BPDCN were complicated by conflict from pathologists, leading to ineffective treatment and a rapid decline in the patient’s condition.
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Single-Center Experience. Clinical Characterization, Mutational Landscape, and Clinical Outcome of Patients Undergoing Hematopoietic Stem Cell Transplantation Intensive Therapy.
Actas Dermosifiliogr
June 2024
Department of Dermatology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain. Electronic address:
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematodermic neoplasm usually involving the skin. In this retrospective case series, 10 cases of BPDCN were identified, 90% of which had skin involvement and exhibited predominantly violaceous nodules and/or bruise-like plaques. Skin lesions showed diffuse or nodular dermal-based infiltrates of intermediate sized blasts with a grenz zone.
View Article and Find Full Text PDFBlastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview.
Hematol Rep
December 2023
Section of Molecular Pathology, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari "Aldo Moro", 70124 Bari, Italy.
Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August-September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor.
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