Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN)

Study Overview
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Brief Summary
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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.

Official Title
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Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN): a Retrospective Study of Patients Diagnosed in France From 2000 to 2013

Conditions
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Blastic Plasmacytoid Dendritic Cell Neoplasm, Hematological Malignancies

Participation Criteria
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Eligibility Criteria
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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 exclusion criteria
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| description of the efficacy of treatments for BPDCN | Measurement of the overall survival for all patients from the date of diagnostic until death or end of data collection | from the diagnosis to death or until June 2013, date of end of data collection | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| description of clinical profiles of patients | presence of cutaneous lesions | from the diagnosis to death or until June 2013, end of data collection | 
| description of biological profiles of patients | immunophenotyping profile | from the diagnosis to death or until June 2013, end of data collection | 
| 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 | 
| survival without events |  | from the diagnosis to death or until June 2013, end of data collection | 
| 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 |