title: Lenalidomide, celecoxib, and azacitidine therapy for blastic plasmocytoid dendritic cell neoplasm: a case report.
abstract: 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.
authors.affiliation: Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
authors.forename: Marta
authors.initials: M
authors.lastname: Garcia-Recio
authors.affiliation: Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
authors.forename: Jordi
authors.initials: J
authors.lastname: Martinez-Serra
authors.affiliation: Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
authors.forename: Leyre
authors.initials: L
authors.lastname: Bento
authors.affiliation: Instituto de Investigación Sanitaria de Palma (IdISPa); Service of Pathology.
authors.forename: Rafael
authors.initials: R
authors.lastname: Ramos
authors.affiliation: Pharmacy Department.
authors.forename: Jordi
authors.initials: J
authors.lastname: Gines
authors.affiliation: Service of Nuclear Medicine, Son Espases University Hospital, Palma de Mallorca, Spain.
authors.forename: Jaime
authors.initials: J
authors.lastname: Daumal
authors.affiliation: Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
authors.forename: Antonia
authors.initials: A
authors.lastname: Sampol
authors.affiliation: Service of Hematology; Instituto de Investigación Sanitaria de Palma (IdISPa).
authors.forename: Antonio
authors.initials: A
authors.lastname: Gutierrez
keywords: COX-2; blastic plasmocytoid dendritic cell neoplasm; elderly; hypomethylating; immunomodulatory