title: Blastic plasmacytoid dendritic cell neoplasm: skin and bone marrow infiltration of three cases and the review of the literature.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a distinct and rare neoplastic entity and was classified as a subgroup of acute myeloblastic leukemia by the WHO in 2008. The median survival of patients was 15.2 months in a large case series. Allogeneic or autologous bone marrow transplantation has been recommended by some reports because of the disease's poor prognosis. We present three patients who presented with both skin and bone marrow infiltration. A 57-year-old man, a 62-year-old woman, a 64-year-old man were admitted to our outpatient clinic because of skin lesions. All of the patient's had bone marrow infiltration with positivity of the CD4, CD56, and CD123 staining. Survival of the patient's were 42, 6 and 12 months, respectively. Two of the patients who presented as blastic form didn't respond to any chemotherapy. BPDCN is a difficult disease to diagnosis and manage. CD4, CD56, CD123, CD303, and T cell leukemia/lymphoma 1. Cutaneous lesions can present as isolated nodules, macules, and disseminated macules and nodules. Positivities are crucial to the diagnosis of the disease in histological examination. Bone marrow infiltration or disease relapse at presentation were related to poor prognosis. Complete immunocytochemical staining must be performed for all patients who have cutaneous lesions with or without blood count abnormalities. Bone marrow (allogeneic or autologous) transplantation should be considered at the first remission.
authors.affiliation: Department of Hematology, Başkent University School of Medicine, Oymaci Sok, No: 7, Altunizade, Istanbul, Turkey.
authors.forename: Figen
authors.initials: F
authors.lastname: Atalay
authors.affiliation: Department of Dermatology, School of Medicine, Baskent University, Istanbul, Turkey.
authors.forename: Gülşen Tükenmez
authors.initials: GT
authors.lastname: Demirci
authors.affiliation: Department of Dermatology, Kocaeli University School of Medicine, Kocaeli, Turkey.
authors.forename: Dilek
authors.initials: D
authors.lastname: Bayramgürler
authors.affiliation: Department of Hematology, Kocaeli University School of Medicine, Kocaeli, Turkey.
authors.forename: Elif Birtaş
authors.initials: EB
authors.lastname: Ateşoğlu
authors.affiliation: Department of Pathology, School of Medicine, Baskent University, Istanbul, Turkey.
authors.forename: Semsi
authors.initials: S
authors.lastname: Yıldız
keywords: Blastic plasmacytoid dendritic cell neoplasm; Bone marrow neoplasms; Skin neoplasms
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