[Clinical Characteristics of CD4^-CD56⁺ Blastic Plasmacytoid Dendritic Cell Neoplasm]

To explore the clinical manifestations, pathological features, immunophenotype, as well as diagnosis, treatment and prognosis of patients with CD4^-CD56⁺ blastic plasmacytoid dendritic cell neoplasm (BPDCN), in order to further understand the rare disease.

The clinical data, laboratory examinations and treatment regimens of two patients with CD4^-CD56⁺ BPDCN in the First Affiliated Hospital of Wannan Medical College were retrospectively analyzed.

The two patients were both elderly males with tumor involved in skin, bone marrow, lymph nodes, etc. Immunohistochemical results of skin lesions showed that both CD56 and CD123 were positive, while CD4, CD34, TdT, CD3, CD20, MPO and EBER were negative. Flow cytometry of bone marrow demonstrated that CD56, CD123, and CD304 were all positive, while specific immune markers of myeloid and lymphoid were negative. Two patients were initially very sensitive to acute lymphoblastic leukemia or lymphomatoid chemotherapy regimens, but prone to rapid relapse. The overall survival of both patients was 36 months and 4 months, respectively.

CD4^-CD56⁺ BPDCN is very rare and easily misdiagnosed as other hematological tumors with poor prognosis. Acute lymphoblastic leukemia or lymphomatoid therapy should be used first to improve the poor prognosis.