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

J Gil-Lianes; P Mozas; T Baumann; A Combalia; C Baliu-Piqué; A García; M Rovira; M López-Guerra; N Villamor; D Colomer; M Rozman; J Esteve; T Estrach

doi:10.1016/j.ad.2023.09.029

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. 2024 Jun 7:S0001-7310(24)00487-3. doi: 10.1016/j.ad.2023.09.029. Online ahead of print.

[Article in English, Spanish]

Authors

J Gil-Lianes¹, P Mozas², T Baumann², A Combalia¹, C Baliu-Piqué¹, A García³, M Rovira², M López-Guerra⁴, N Villamor⁴, D Colomer⁴, M Rozman⁵, J Esteve², T Estrach⁶

Affiliations

¹ Department of Dermatology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
² Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
³ Department of Pathology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
⁴ Hematopathology Section, Pathology Department, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain.
⁵ Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain; Hematopathology Section, Pathology Department, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
⁶ Department of Dermatology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain. Electronic address: [email protected].

PMID: 38852841
DOI: 10.1016/j.ad.2023.09.029

Abstract

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. Tumor immunophenotyping was CD4(+), CD56(+), CD123(+) and CD303(+). The most frequently mutated genes according to targeted next-generation sequencing were TET2 (3/7) and NRAS (2/7). Multiagent chemotherapy (CT) was administered as first-line therapy, and a total of 5 patients underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). Better outcomes were observed in younger patients and those treated with acute lymphoblastic leukemia (ALL)-like CT followed by allo-HSCT. This study shows the clinical range of cutaneous lesions of BPDCN. Despite the absence of a gold standard therapy, patients treated with myeloablative intensive regimens and allo-HSCT seems to have a more favorable prognosis.

Keywords: Blastic plasmacytoid dendritic cell neoplasm; Cutaneous lymphomas; Hematologic neoplasm; Hematopoietic stem cell transplantation; Linfoma cutáneo; Neoplasia blástica de células dendríticas plasmocitoides; Neoplasia hematológica; Next-generation sequencing; Trasplante alogénico de progenitores hematopoyéticos.

Publication types

Case Reports