Blastic plasmacytoid dendritic cell neoplasm: Two cases and literature review

doi:10.3969/j.issn.1004-583X.2024.03.010

Abstract

Abstract:

Objective To explore the clinical and pathological characteristics, treatment, and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Methods Based on literature review, we retrospectively analyzed clinical manifestations, histological morphology, immune phenotype, treatment plan, and prognosis of two patients with BPDCN. Results The tissue morphology of both cases was consistent with the pathological changes in BPDCN. Immunohistochemical markers cluster of differentiation (CD)4, CD56, CD123, T-cell leukemia-1 (TCL1) were positively stained, and lymphoid, myeloid, and NK cell sources were ruled out. Epstein-Barr virus-encoded RNA (EBER) in situ hybridization results of both cases were negative. Case 1 involved bone marrow and lymph nodes, received symptomatic supportive treatment, and died 2 months later. Case 2 was managed by 8 cycles of chemotherapy with the cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) regimen. The condition of Case 2 progressed at 6 months with multiple rubella throughout the body. Later, gemcitabine, oxaliplatin, and pemetrexed chemotherapy were administered for 9 cycles. Currently, the condition of Case 2 was stable. Conclusion BPDCN often have skin symptoms as the first symptom and unique clinical and pathological characteristics. Diagnosis of BPDCN relies on histology and immunophenotype. There is currently no effective treatment plan, leading to a poor prognosis of BPDCN.

Key words: blastic plasmacytoid dendritic cell neoplasm, pathology, clinical, immunophenotyping, treatment

CLC Number:

R733

Ren Lei, Liu Ye, Bao Shuyou, Li Kuifang. Blastic plasmacytoid dendritic cell neoplasm: Two cases and literature review[J]. Clinical Focus, 2024, 39(3): 253-258.

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URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2024.03.010

https://huicui.hebmu.edu.cn/EN/Y2024/V39/I3/253

Figures/Tables 10

Fig.1 Skin lesion located on the forehead, with slightly raised and hard light purple-red nodule

Fig.2 Invasive growth of tumor cells in dermis (HE, low power field)

Fig.3 Infiltration of tumor cells into striated muscle tissue (HE, middle power field)

Fig.4 No epidermal affinity (HE, middle power field)

Fig.5 High power microscopic showed that the cells are single, small to medium large, with extremely irregular and oval karyotype, small nucleolus, mitotic phase, and light staining of the cytoplasm (HE staining, High power microscopic)

Fig.6 CD56 positive expression (immunohistochemical staining, Polymer Conjugate method)

Fig.7 CD4 positive expression (immunohistochemical staining, Polymer Conjugate method)

Fig.8 CD123 positive expression (immunohistochemical staining, Polymer Conjugate method)

Fig. 9 Skin lesions on the back a. Back skin before lesion progression; b. Multiple light gray to light red plaques can be seen on the skin of the back after lesion progression

Fig.10 Skin lesions on the arm a.Arm skin before lesion progression;b.Arm skin after lesion progression, multiple reddish patches are seen

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