[Clinical characteristics of 6 patients with blastic plasmacytoid dendritic cell neoplasm]
X Han1, M Q Ouyang, Q Pei
1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing 100730, China.
Insights
Blastic plasmacytoid dendritic cell neoplasm is an aggressive cancer. Initial symptoms often include skin lesions, and treatment with acute lymphoblastic leukemia-type chemotherapy can achieve remission, but prognosis remains poor.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy.
- It is characterized by distinctive clinical, morphological, and immunophenotypic features.
Purpose of the Study:
- To investigate the clinical characteristics, treatment strategies, and prognosis of BPDCN.
- To analyze patient outcomes following chemotherapy.
Main Methods:
- Retrospective analysis of clinical data from 6 BPDCN patients diagnosed between January 2008 and May 2016.
- Evaluation of immunophenotype, treatment response, and survival data.
Main Results:
- Skin lesions were the most common initial symptom (6/6).
- Bone marrow involvement (5/6), lymphadenectasis (4/6), splenomegaly (4/6), and hepatomegaly (3/6) were frequent.
- Characteristic immunophenotype: CD4+, CD56+, CD123+.
- Complete remission (CR) achieved in 4/6 patients treated with acute lymphoblastic leukemia (ALL)-type chemotherapy.
- Median progression-free survival was 7 months, and median overall survival was 9 months.
- 3 patients died within the first year, primarily due to relapse or disease progression.
Conclusions:
- BPDCN is a highly aggressive neoplasm with frequent skin and bone marrow involvement.
- The immunophenotype CD4, CD56, and CD123 is characteristic.
- Limited effective standard therapies contribute to a poor prognosis.
Abstract:
Objective: To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm. Method: Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed. Results: Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) . In addition, extra-nodal involvement except skin was also observed, including breast (1/6) , maxillary sinus (1/6) , vertebrae (1/6) , and central nervous system (1/6) . Characteristic immunophenotype, CD4, CD56, and CD123 were all positive. All these patients were treated with acute lymphoblastic leukemia type (ALL-type) chemotherapy and complete remission (CR) were reached in 4 patients. The median follow-up was 9.5 (7-37) months, median progression free survival was 7 months; while median overall survival was 9 months. A total of 3 patients died during the follow-up, which were all happened in the first year after diagnosis, and all resulted from the relapse or disease progression. Conclusion: Blastic plasmacytoid dendritic cell neoplasm is highly aggressive, in which the skin lesions are always manifested as initial symptoms, and bone marrow involvement, lymphadenectasis, splenomegaly, and hepatomegaly is also common. Characteristic immunophenotype include the positivity of CD4, CD56, and CD123. Effective and standard therapy is limited in this disease, which indicates the poor prognosis.
