title: Leukemic manifestation of blastic plasmacytoid dendritic cell neoplasm lacking skin lesion : a borderline case between acute monocytic leukemia.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with a poor prognosis. We encountered a unique case of BPDCN that was leukemic at presentation without skin lesion and expressed CD33 antigen. A 74-year-old man was admitted because of dyspnea. Physically, hepatosplenomegaly, but not skin lesions and superficial lymph node swelling, was noted. The white blood count was 33.6 × 10(9)/L with 19% giant abnormal cells. These cells were positive for CD4, CD86, CD123 (bright), BDCA-2, and HLA-DR, but negative for CD1a, CD3, CD11b, CD11c, CD13, CD14, CD19, CD64, and CD68. From these findings, a diagnosis of BPDCN was made. In terms of unusual expression, these tumor cells were positive for CD33 but negative for CD56. The karyotype was 47, XY, t(6;8) (p21;q24), + r. We performed combination chemotherapy (Ara-C + VP-16 + MIT), which resulted in a marked reduction of tumor cells and improvement of the dyspnea. On day 16, however, he died of sepsis due to Bacillus cereus. The clinical picture of this patient is unusual and may provide new information on the clinicopathology of BPDCN.
authors.affiliation: Departments of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Kobe, Japan. tytakiuchi@yahoo.com.jp
authors.forename: Yoko
authors.initials: Y
authors.lastname: Takiuchi
authors.forename: Hayato
authors.initials: H
authors.lastname: Maruoka
authors.forename: Kazunari
authors.initials: K
authors.lastname: Aoki
authors.forename: Aiko
authors.initials: A
authors.lastname: Kato
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Ono
authors.forename: Seiji
authors.initials: S
authors.lastname: Nagano
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Arima
authors.forename: Daichi
authors.initials: D
authors.lastname: Inoue
authors.forename: Minako
authors.initials: M
authors.lastname: Mori
authors.forename: Sumie
authors.initials: S
authors.lastname: Tabata
authors.forename: Soshi
authors.initials: S
authors.lastname: Yanagita
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsushita
authors.forename: Mari
authors.initials: M
authors.lastname: Nishio
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
authors.forename: Kiminari
authors.initials: K
authors.lastname: Ito
authors.forename: Haruyuki
authors.initials: H
authors.lastname: Fujita
authors.forename: Norimitsu
authors.initials: N
authors.lastname: Kadowaki
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000276:immunology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003713:Dendritic Cells / Q000187:drug effects / Q000473:pathology*; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000473:pathology; D017809:Fatal Outcome; D006680:HLA Antigens / Q000276:immunology; D019337:Hematologic Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D006529:Hepatomegaly / Q000473:pathology; D006801:Humans; D007948:Leukemia, Monocytic, Acute / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D013163:Splenomegaly / Q000473:pathology; D014178:Translocation, Genetic
chemical_list: D015703:Antigens, CD; D006680:HLA Antigens