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Correspondence
Blastic plasmacytoid dendritic cell neoplasm with EWSR1 gene rearrangement
  1. Qinghua Cao1,
  2. Fang Liu2,
  3. Gang Niu3,
  4. Ling Xue1,
  5. Anjia Han1
  1. 1Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
  2. 2Department of Oncology, NanFang Hospital, Southern Medical University, Guangzhou, China
  3. 3Department of Gynaecology and Obstetrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
  1. Correspondence to Dr Anjia Han, Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University, 58, Zhongshan Road II, Guangzhou 510080, China; hananjia{at}mail.sysu.edu.cn

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Introduction

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare form of haematologic neoplasm, which was previously referred to as blastic natural killer-cell lymphoma, agranular CD4 natural killer cell leukaemia or agranular CD4/CD56 haematodermic neoplasm. It1 has recently been recognised as a distinct entity and a rare subgroup of acute myeloid leukaemia (AML) and a related precursor neoplasm in WHO classification of tumours of haematopoietic and lymphoid tissues (2008).

Ewing sarcoma breakpoint region 1(EWSR1) has a large number of fusion partners, mainly associated with the pathogenesis of Ewing sarcoma family tumours. Herein, we first reported one BPDCN case that showed positive EWSR1 gene rearrangement.

Clinical findings

The patient was a 5-year-old girl. She complained of painless purple skin papules for 6 months. The lesion increased to 5 cm×4 cm in size after 3 monthswithout involvement of lymph node or other organs. The examination of peripheral blood and bone marrow revealed …

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