Clinical Characteristics and Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm.
10.19746/j.cnki.issn.1009-2137.2023.01.040
- Author:
Xiao-Li ZHANG
1
;
Bing LIU
1
;
Nan LI
1
;
Lu-Ke LI
2
;
Xuan-Jing JI
1
;
Xue-Fang ZHOU
1
;
Min-Fang WANG
1
;
Hui-Li XU
3
Author Information
1. Hematology Department, Pingdingshan First People's Hospital, Pingdingshan 467021, Henan Province, China.
2. Hematology Department, General Hospital of Pingmei Shenma Medical Group, Pingdingshan 467002, Henan Province, China.
3. Hematology Department, Pingdingshan First People's Hospital, Pingdingshan 467021, Henan Province, China,E-mail: pdszyb@126.com.
- Publication Type:Journal Article
- Keywords:
blastic plasmacytoid dendritic cell neoplasm;
chemotherapy;
clinical features;
immunophenotyping
- MeSH:
Humans;
Dendritic Cells;
Retrospective Studies;
Skin Neoplasms/pathology*;
Antineoplastic Agents/therapeutic use*;
Bone Marrow/pathology*;
Myeloproliferative Disorders;
Hematologic Neoplasms/drug therapy*
- From:
Journal of Experimental Hematology
2023;31(1):254-260
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).
METHODS:The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively.
RESULTS:4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine.
CONCLUSION:The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.
