母细胞性浆细胞样树突细胞肿瘤4例临床病理分析

Blastic plasmacytoid dendritic cell neoplasm:a clinicopathologic study of 4 cases

目的探讨母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床病理学特征及诊断与鉴别诊断。方法对4例BPDCN的临床病理学特征、免疫表型以及治疗和预后情况进行分析,并复习相关文献。结果 4例患者均为男性,发病年龄20～70岁;2例表现为淋巴结肿大,2例示多发性皮肤斑块或结节。活检组织形态学显示,淋巴结及真皮和皮下由弥漫浸润的小～中等大小幼稚肿瘤细胞浸润,细胞质稀少,核形不规则,染色质细腻,核仁不明显。免疫组化示肿瘤细胞CD4、CD56、CD123、TCL-1和S-100(+)。结论 BPDCN是一种罕见的高度恶性的淋巴造血系统肿瘤,多数病例发生于老年患者,常见皮肤累及;少量病例也可发生于年轻患者或无皮肤受累者。即使给予高强度化疗和同种异体骨髓移植,预后仍很差。

Objective To investigate the features, pathological diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm （BPDCN）. Methods The clinical and histological findings, immunophenotype, treatment and prognosis in 4 cases of BPDCNs were evaluated with review of the relevant literatures. Results All 4 patients were male, and 2 patients presented as lymphoadenopathy, 2 patients presented as multiple cutaneous lesions, with age from 20 to 70 years. Microscopically, the tumors were composed of diffuse blasts with small to medium size, scant cytoplasm, highly irregular nuclear contours, dispersed nuclear chromatin and faint indistinct nucleolus in lymph nodes （ cases 1 and 2 ） or dermis and subcutaneous tissue （cases 3 and 4）~ Immunophenotypically, the tumor cells were positive for CD4, CD56, CD123, TCL-1 and $100. Conclusions BPDCN is a rare high grade hematopoietic neoplasm with very poor survival even though treated with multiagents chemotherapy and allogeneic bone marrow transplantation. Skin involvement is very common, but young patients with BPDCNs and tumors only with extracutaneous presentations can be seen in rare cases.