Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement

Background: We describe a patient with blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement and the outcome of venetoclax use in this setting. Case presentation: A 54-year-old Caucasian male was referred to the Haematology Unit with an enlarged inguinal lymph node whi...

ver descrição completa

Detalhes bibliográficos
Autores: Albiol, Nil|||0000-0003-2942-1362, Novelli, Silvana|||0000-0001-8750-0195, Mozos, Ana|||0000-0002-3350-1827, Pratcorona, Marta|||0000-0001-6375-596X, Martino Bofarull, Rodrigo|||0000-0001-5143-4042, Sierra, Jorge|||0000-0002-7966-0356
Formato: artículo
Fecha de publicación:2021
País:España
Recursos:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:269708
Acesso em linha:https://ddd.uab.cat/record/269708
https://dx.doi.org/urn:doi:10.1186/s13256-021-02939-7
Access Level:acceso abierto
Palavra-chave:Blastic plasmacytoid dendritic cell neoplasm
Bcl-2
Venetoclax
BPDCN
Descrição
Resumo:Background: We describe a patient with blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement and the outcome of venetoclax use in this setting. Case presentation: A 54-year-old Caucasian male was referred to the Haematology Unit with an enlarged inguinal lymph node which was diagnostic of a blastic plasmacytoid dendritic cell neoplasm. The staging revealed disseminated disease (skin, visceral, lymph nodes, and bone marrow). He received chemotherapy with an acute myeloid leukaemia-like regime. Afterwards, he underwent allogeneic haematopoietic stem cell transplantation, though it was not successful, showing a relapse 14 months later with hepatic and central nervous system dissemination. Intrathecal chemotherapy was administered, and venetoclax (anti-bcl2 agent) was started in an off-label indication based on most recent literature. The disease halted its course for 3 months. In the end, the patient's disease progressed and so he succumbed due to infectious complications. Conclusions: Venetoclax monotherapy seems not enough to control the disease progression under CNS involvement and other treatments should be investigated.