Molina Castro Diego, Perilla Suárez Oliver, Cuervo-Sierra Jorge, Moreno Alexandra
Internal Medicine, Hospital San Vicente Fundación, Medellín, COL.
Hematology, Universidad de Antioquia, Medellín, COL.
Cureus. 2022 Apr 6;14(4):e23888. doi: 10.7759/cureus.23888. eCollection 2022 Apr.
Blastic plasmacytoid dendritic cell neoplasm is a rare hematologic neoplasm characterized by cutaneous, hematologic, and central nervous system (CNS) involvement with poor prognosis. Diagnosis is made by flow cytometry, although there are no specific markers, making its diagnosis challenging. So far, with the available evidence, acute lymphoid leukemia-type schemes and consolidation with allogeneic transplant seem to become the first-line therapy. With its characterization, new therapies directed toward CD123 and the anti-apoptotic protein Bcl-2 have appeared to prolong the survival of these patients. We present a case of a 27-year-old male patient diagnosed with blastic plasmacytoid dendritic cell neoplasm with unusual CNS manifestations and without skin involvement who achieved complete remission with venetoclax and improvement of neurological symptoms, making him a candidate for hematopoietic stem cell transplant.
母细胞性浆细胞样树突状细胞肿瘤是一种罕见的血液肿瘤，其特征为皮肤、血液和中枢神经系统（CNS）受累，预后较差。诊断通过流式细胞术进行，尽管没有特异性标志物，这使得其诊断具有挑战性。到目前为止，根据现有证据，急性淋巴细胞白血病类型的方案以及异基因移植巩固治疗似乎成为一线治疗方法。随着对其特征的了解，针对CD123和抗凋亡蛋白Bcl-2的新疗法似乎延长了这些患者的生存期。我们报告一例27岁男性患者，诊断为母细胞性浆细胞样树突状细胞肿瘤，有不寻常的中枢神经系统表现且无皮肤受累，该患者使用维奈克拉实现了完全缓解并改善了神经症状，使其成为造血干细胞移植的候选者。
