Garcia-Recio Marta, Martinez-Serra Jordi, Bento Leyre, Ramos Rafael, Gines Jordi, Daumal Jaime, Sampol Antonia, Gutierrez Antonio
Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
Instituto de Investigación Sanitaria de Palma (IdISPa); Service of Pathology.
Onco Targets Ther. 2016 Sep 7;9:5507-11. doi: 10.2147/OTT.S107893. eCollection 2016.
Blastic plasmocytoid dendritic cell neoplasm is characterized by aggressive behavior with a tendency for systemic dissemination and a predilection for skin, lymph nodes, soft tissues, peripheral blood, or bone marrow. It usually occurs in elderly patients with a mean age between 60 and 70 years. Despite initial response to chemotherapy, the disease regularly relapses with a short median overall survival. Better outcomes have been reported with high-dose acute leukemia-like induction chemotherapy followed by consolidation with allogeneic hematopoietic stem cell transplantation. However, elderly patients are not candidates for intensive therapy or allogeneic stem cell transplantation. So, new active and tolerable drugs are needed. Our case illustrates that one cycle of lenalidomide and celecoxib provides at least a partial cutaneous and hematologic response, but this regimen was discontinued due to toxicity and followed by a consolidation/maintenance phase with azacitidine, thus achieving a final complete response with a much higher than expected progression-free and overall survival in an elderly patient with comorbidities. This information may be useful in the design of treatment approaches for elderly patients with blastic plasmocytoid dendritic cell neoplasm. However, it should be confirmed in clinical trials as well as by optimizing the induction and extending the consolidation/maintenance period to avoid early relapses after discontinuation and improve progression-free survival.
母细胞性浆细胞样树突状细胞肿瘤的特点是侵袭性强，有全身播散倾向，好发于皮肤、淋巴结、软组织、外周血或骨髓。它通常发生在平均年龄为60至70岁的老年患者中。尽管对化疗最初有反应，但该疾病经常复发，总体生存期中位数较短。据报道，采用大剂量急性白血病样诱导化疗，随后进行异基因造血干细胞移植巩固治疗，可取得更好的疗效。然而，老年患者不适合进行强化治疗或异基因干细胞移植。因此，需要新的有效且耐受性良好的药物。我们的病例表明，来那度胺和塞来昔布一个周期的治疗至少提供了部分皮肤和血液学反应，但由于毒性该方案被停用，随后进入阿扎胞苷巩固/维持阶段，从而在一名患有合并症的老年患者中实现了最终的完全缓解，无进展生存期和总生存期远高于预期。这些信息可能有助于设计针对老年母细胞性浆细胞样树突状细胞肿瘤患者的治疗方法。然而，这需要在临床试验中得到证实，同时通过优化诱导治疗和延长巩固/维持期来避免停药后的早期复发并提高无进展生存期。
