Leukemic Manifestation of Blastic Plasmacytoid Dendritic Cell Neoplasm Lacking Skin Lesion : A Borderline Case between Acute Monocytic Leukemia

Yoko Takiuchi; Hayato Maruoka; Kazunari Aoki; Aiko Kato; Yuichiro Ono; Seiji Nagano; Hiroshi Arima; Daichi Inoue; Minako Mori; Sumie Tabata; Soshi Yanagita; Akiko Matsushita; Mari Nishio; Yukihiro Imai; Yukihiro Imai; Kiminari Ito; Haruyuki Fujita; Norimitsu Kadowaki; Takayuki Ishikawa; Takayuki Takahashi

doi:10.3960/jslrt.52.107

Case Study

Leukemic Manifestation of Blastic Plasmacytoid Dendritic Cell Neoplasm Lacking Skin Lesion : A Borderline Case between Acute Monocytic Leukemia

Yoko Takiuchi, Hayato Maruoka, Kazunari Aoki, Aiko Kato, Yuichiro Ono, Seiji Nagano, Hiroshi Arima, Daichi Inoue, Minako Mori, Sumie Tabata, Soshi Yanagita, Akiko Matsushita, Mari Nishio, Yukihiro Imai, Yukihiro Imai, Kiminari Ito, Haruyuki Fujita, Norimitsu Kadowaki, Takayuki Ishikawa, Takayuki Takahashi

Author information

Yoko Takiuchi
Departments of Hematology and Clinical Immunology
Hayato Maruoka
Departments of Clinical Laboratory
Kazunari Aoki
Departments of Hematology and Clinical Immunology
Aiko Kato
Departments of Hematology and Clinical Immunology
Yuichiro Ono
Departments of Hematology and Clinical Immunology
Seiji Nagano
Departments of Hematology and Clinical Immunology
Hiroshi Arima
Departments of Hematology and Clinical Immunology
Daichi Inoue
Departments of Hematology and Clinical Immunology
Minako Mori
Departments of Hematology and Clinical Immunology
Sumie Tabata
Departments of Hematology and Clinical Immunology
Soshi Yanagita
Departments of Hematology and Clinical Immunology
Akiko Matsushita
Departments of Hematology and Clinical Immunology
Mari Nishio
Departments of Clinical Pathology, Kobe City Medical Center General Hospital
Yukihiro Imai
Departments of Clinical Pathology, Kobe City Medical Center General Hospital
Yukihiro Imai
Departments of Clinical Pathology, Kobe City Medical Center General Hospital
Kiminari Ito
Departments of Foundation of Biomedical Research and Innovation
Haruyuki Fujita
Departments of Hematology/Oncology, Graduate School of Medicine
Norimitsu Kadowaki
Departments of Hematology/Oncology, Graduate School of Medicine
Takayuki Ishikawa
Departments of Hematology/Oncology, Graduate School of Medicine
Takayuki Takahashi
Departments of Hematology and Clinical Immunology

Keywords: blastic plasmacytoid dendritic cell neoplasm, leukemic manifestation, skin lesion, CD33, CD56

JOURNAL FREE ACCESS

2012 Volume 52 Issue 2 Pages 107-111

DOI https://doi.org/10.3960/jslrt.52.107

Details

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 × 10⁹/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. [J Clin Exp Hematopathol 52(2) : 107-111, 2012]

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