title: Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-Color flow cytometry diagnosis and HyperCVAD therapy.
abstract: Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo-HCT). For a median follow up of 13.3 months, the 1-year disease free survival and overall survival were 56 and 67%, respectively. An accurate diagnosis of BPDCN can be made by 10-color FCM using a four-tube AL panel demonstrating a characteristic pattern of antigen expression. Front-line induction chemotherapy with HyperCVAD can yield high remission rates, but allo-HCT is required for long-term durable remissions.
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Uday
authors.initials: U
authors.lastname: Deotare
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Karen W L
authors.initials: KW
authors.lastname: Yee
authors.affiliation: Department of Biostatistics, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Lisa W
authors.initials: LW
authors.lastname: Le
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: Anna
authors.initials: A
authors.lastname: Porwit
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: Anne
authors.initials: A
authors.lastname: Tierens
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: Rumina
authors.initials: R
authors.lastname: Musani
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Barth
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: Emina
authors.initials: E
authors.lastname: Torlakovic
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Aaron
authors.initials: A
authors.lastname: Schimmer
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Andre C
authors.initials: AC
authors.lastname: Schuh
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.|Department of Medical Oncology and Hematology, Cancer Care Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: Matthew
authors.initials: M
authors.lastname: Seftel
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Mark D
authors.initials: MD
authors.lastname: Minden
authors.affiliation: Leukemia Program, Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
authors.forename: Vikas
authors.initials: V
authors.lastname: Gupta
authors.affiliation: Department of Pathobiology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Hyjek
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003713:Dendritic Cells / Q000187:drug effects / Q000473:pathology*; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010954:Plasmacytoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D012189:Retrospective Studies; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide