目的 学习母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床病理及免疫表型特征,总结该少见肿瘤的病理诊断经验。方法 回顾分析2例BPDCN患者临床资料,通过苏木素-伊红(HE)染色分析肿瘤组织及细胞形态,通过免疫组织化学染色分析肿瘤免疫表型,通过流式细胞学检测骨髓有无肿瘤侵犯,并结合文献分析。结果 本报道中1例为97岁女性,临床以皮肤瘀斑结节为首发症状,肿瘤细胞真皮内弥漫浸润,不侵犯表皮,细胞中等大小,核形不规则,核仁不明显。另1例为69岁男性,临床以淋巴结肿大为首发症状,淋巴结结构完全破坏,肿瘤细胞弥漫浸润,细胞呈中等大小的母细胞样,核仁明显。2例免疫表型均表达CD123、CD4、CD56、TDT,不表达B系、T系淋巴细胞及髓系标志物,肿瘤均累及骨髓。结论 BPDCN是一种罕见的淋巴造血肿瘤,临床常以皮肤病变或淋巴结肿大为首发症状,临床过程具高度侵袭性,通常伴有骨髓侵犯。该肿瘤需与具有母细胞形态的淋巴系肿瘤和白血病相鉴别,诊断需结合临床信息、HE形态及免疫组化结果综合判断。

Objective To summarize the diagnostic experiences of blastic plasmacytoid dendritic cell neoplasm (BPDCN) based on the study of its clinicopathological features and immunophenotypes. Methods The clinical data of 2 patients with BPDCN were collected, the structure alteration and cell morphology were observed by HE staining, the immunophenotype of tumor cells were studied by immunohistochemistry staining and flow cytometry was adopted to confirm the bone marrow involvement. Results Two patients, one of whom was a 97 year-old female, presented with cutaneous ecchymosis nodules as the first symptom. The epidermis, but not the dermal, was diffusedly infiltrated by tumor cells, which were medium-sized with irregular nuclei without prominent nucleoli. The other case was a 69 year-old male with lymph node enlargement as the first symptom. The skin was normal, but the lymph nodes were invasively destroyed by tumor cells, which were medium-sized blast-like with prominent nucleoli. The immunophenotypes of the two patients were both positive for CD123, CD4, CD56 and TDT, but negative for B, T lymphocyte derived and myeloid origin markers, both of which involved bone marrow. Conclusions BPDCN is a rare form of hematological neoplasm, skin symptoms or lymph node enlargement may be presented as the initial symptom, the clinical course were highly aggressive with high frequency of bone marrow involvement. The blastic-like lymphoma and leukemia entities should be considered into account for differential diagnose. The precise diagnosis of BPDCN should be established by integrating histomorphology, immunophenotype and clinical presentation information comprehensively.

       近年来浆细胞性乳腺炎的发病率逐渐升高，已占乳腺疾病的4%~5%，该病易反复发作，经久不愈，抗生素、糖皮质激素、抗结核药、他莫昔芬等治疗效果不明显，单纯手术切除如切除范围小极易复发，而切除范围大则乳腺外形变化较大，严重者甚至需要切除乳腺。乳腺外形的缺损改变对患者身心健康造成伤害。随着研究不断深入，采用中西医结合治疗浆细胞性乳腺炎，可取得较好的治疗效果，降低复发率，而且对乳腺外观无影响。该文通过对文献的整理，对中西医结合治疗浆细胞性乳腺炎进行论述。

       In recent years，the incidence of plasma cell mastitis has gradually increased，accounting for 4 % ~ 5 % of breast diseases．The disease is prone to repeated attacks and unhealed for a long time．The treatment effect of antibiotics，glucocorticoids，anti-tuberculosis drugs and tamoxifen is not obvious．Simple surgical  resection is easy to  relapse if the  resection range is small，while the shape of the breast changes greatly，if the resection range is large．And even the breast needs to be removed in severe cases．The defect change of breast shape is harmful to the physical and mental health of patients．With the deepening of research，the combination of traditional Chinese and Western medicine in the treatment of plasma cell mastitis can achieve better therapeutic effect，reduce the recurrence rate，and has no effect on the appearance of breast．This article discusses the treatment of plasma cell mastitis with integrated traditional Chinese and Western medicine by sorting out the literature．