Hernández Danay Caballero, Rueda Darío Álvaro, Rapan Leticia, Iastrebner Marcelo, Sorrentino Miguel
Bone Marrow Transplant Service, Sanatorio Sagrado Corazón, Buenos Aires, CP 1039, Argentina.
Internal Medicine Service, Sanatorio Sagrado Corazón, Buenos Aires, CP 1039, Argentina.
Ecancermedicalscience. 2024 Nov 29;18:1806. doi: 10.3332/ecancer.2024.1806. eCollection 2024.
Plasmacytoid blast dendritic cell neoplasm is a rare subtype of acute leukaemia that represents less than 1% of haematologic neoplasms. It is characterised by skin involvement and leukaemic dissemination in the rest of the body. The immunophenotype is represented by the expression of CD4, CD56 and CD123. Due to its low incidence, there is no standardised treatment. For most authors, acute lymphoblastic leukaemia) regimens with or without consolidation with allogeneic transplantation seem to be the most appropriate. We present the case of a 31-year-old male with a history of von Willebrand's disease, who was diagnosed with plasmacytoid blast dendritic cell neoplasm with central nervous system involvement. After receiving first-line immunopolychemotherapy with rituximab, the patient achieved complete haematologic remission with the high-dose ara-C regimen. Subsequently, he consolidated with allogeneic haploidentical transplantation.
浆细胞样原始树突状细胞肿瘤是急性白血病的一种罕见亚型，占血液系统肿瘤的比例不到1%。其特征是皮肤受累以及白血病在身体其他部位的播散。免疫表型表现为CD4、CD56和CD123的表达。由于其发病率低，尚无标准化治疗方案。对于大多数作者而言，采用或不采用异基因移植巩固治疗的急性淋巴细胞白血病方案似乎是最合适的。我们报告一例31岁男性病例，该患者有血管性血友病病史，被诊断为伴有中枢神经系统受累的浆细胞样原始树突状细胞肿瘤。在接受了含利妥昔单抗的一线免疫多药化疗后，患者采用大剂量阿糖胞苷方案实现了完全血液学缓解。随后，他接受了异基因单倍体相合移植巩固治疗。
