Blastic Plasmacytoid Dendritic Cell Neoplasm, Hematological Malignancies
Conditions
Brief summary
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease characterized by an aggressive clinical behavior and a poor prognosis. It predominantly affects elderly males with an average age of 67 years at diagnosis and the affected organs are usually the skin, bone marrow, lymph nodes and the central nervous system. Patients with BPDCN have poor outcomes with median overall survival (OS) ranging in the largest series of patients from 8 to 12 months. Patient care must be defined in this pathology. Despite 40%-90% complete remission (CR) rates after initial chemotherapy, relapses are almost inevitable. The investigators have developed a national network to collect clinical and biological data of French patients diagnosed with BPDCN.
Interventions
None listed
Sponsors
Study design
Eligibility
Inclusion criteria
* Patients diagnosed with blastic plasmacytoid dendritic cell neoplasm (BPDCN) from January 2000 to June 2013 in France. * Diagnosis should be established by hematology laboratory of French blood Agency of Bourgogne Franche-Comté and/or by anatomopathological analysis (realized in local centres or by Tony Petrella at Dijon University Hospital) according to phenotypic and anatomopathological criteria published in the literature.
Exclusion criteria
* No
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| description of the efficacy of treatments for BPDCN | from the diagnosis to death or until June 2013, date of end of data collection | Measurement of the overall survival for all patients from the date of diagnostic until death or end of data collection |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| description of biological profiles of patients | from the diagnosis to death or until June 2013, end of data collection | immunophenotyping profile |
| complete remission rate | from the diagnosis to death or until June 2013, end of data collection | — |
| mean duration of the first remission | from the diagnosis to death or until June 2013, end of data collection | — |
| description of clinical profiles of patients | from the diagnosis to death or until June 2013, end of data collection | presence of cutaneous lesions |
| non-responders rate | from the diagnosis to death or until June 2013, end of data collection | — |
| time interval between two treatments | from the diagnosis to death or until June 2013, end of data collection | — |
| mortality without relapse | from the diagnosis to death or until June 2013, end of data collection | — |
| survival without events | from the diagnosis to death or until June 2013, end of data collection | — |