Combination Chemotherapy in Patients With Newly Diagnosed BPDCN

Inclusion Criteria: * Newly diagnosed BPDCN established by a blood or bone marrow immunophenotypic diagnosis by flow cytometric and/or by the anatomic pathology study of a skin biopsy using validated diagnostic criteria (Swerdlow SH CE et al., World Health Organisation Classification of Tumors, 2008; Garnache-Ottou et al., 2009; Angelot et al., 2012; Julia et al., 2014) or patients with confirmed isolated skin lesion. * 18 years of age or older * No prior cytotoxic therapy except \<2 week of corticosteroids or hydroxyurea * ECOG ≤2 * Written informed consent * Affiliation to the French social security scheme Exclusion Criteria: * Cardiac contra-indication to anthracyclines: cardiac dysfunction events (NYHA grade 3 or 4 and/or LVEF\<50%) * Hepatocellular abnormalities except if considered related to the BPDCN: 1. ASAT (SGOT) and/or ALAT (SGPT) \> 5 x ULN 2. Total bilirubin ≥ 2.5 x ULN * Creatinine level \>1.5x ULN or creatinine clearance (MDRD)\<50 mL/mn * Prior thrombotic event * Active hepatitis B or C virus infection * HIV positive * Serious medical or psychiatric illness that could interfere with the completion of treatment * Known allergy to any of the study medications, their analogues, or excipients in the various formulations of any agent. * Pregnant and lactating female patients * Patients diagnosed with or treated for another malignancy within 2 years before study enrollment or with residual disease (basal cell carcinoma or cervical carcinoma in situ patients may be enrolled if they have undergone complete resection)