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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.
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 months without involvement of lymph node or other organs. The examination of peripheral blood and bone marrow revealed normal findings.
Microscopically, the …