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

Centre Hospitalier Universitaire de Besancon86 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Blastic Plasmacytoid Dendritic Cell Neoplasm Hematological Malignancies

Eligibility

Sex: ALL

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

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

Time frame: from the diagnosis to death or until June 2013, date of end of data collection