Clinical features of blastic plasmacytoid dendritic neoplasm in Chile: report of 10 cases

• Published in: Revista medíca de Chile 2023-09, Vol.151 (9), p.1201
• Main Authors: Micolich, Vicente, Peña, Camila, Figueroa, Fernanda, Briones, Vania, Vidal, Catalina, Chandia, Mauricio
• Format: Article
• Language: Spanish
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Chile - epidemiology; Cyclophosphamide - therapeutic use; Dendritic Cells - pathology; Female; Hematologic Neoplasms - pathology; Humans; Male; Middle Aged; Prednisone - therapeutic use; Retrospective Studies; Skin Neoplasms - pathology; Vincristine - therapeutic use; Young Adult
• ISSN: 0717-6163; 0717-6163
• DOI: 10.4067/s0034-98872023000901201

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor with a dismal prognosis, with isolated case reports in Chile. The BPDCN can present skin and bone marrow compromise, and its diagnosis is frequently confused with other pathologies. This study aimed to evaluate the clinical and immunophenotypical features of BPDCN in the Chilean population. We performed a retrospective study from 2013 to 2021 in clinical records of 2 public Chilean referral hospitals, including ten patients, 80% male, with a median age of 66 years (15-81). The most frequent initial referral diagnoses were T-cell lymphoma (4/10) and acute myeloblastic leukemia (3/10). Seven patients presented skin and bone marrow involvement; we found a lower frequency of adenopathies (5/10), splenomegaly (2/10), and hepatomegaly (2/10). The complete blood count revealed anemia and leukopenia, with blasts in 5/10. Nine patients received induction therapy. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) was indicated in 8/10 cases with remission in 5/8, and 1 patient received HyerCVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone, methotrexate, cytarabine) and an allogeneic bone marrow transplant. The median survival was 10 months (95% CI 4.2-15.8 months) with 9/10 deaths. Relapse in the central nervous system was documented in 2 cases. Our study found that BPDCN, a rare pathology in the Chilean population, shows a similar clinical presentation compared to previous studies. It is susceptible to respond to initial systemic and intrathecal chemotherapy.