目的 通过学习1例少见的组织学特征与IgG4相关性淋巴结病类似的浆细胞型特发性多中心型Castleman病（PC-iMCD）,总结两种易误诊疾病的鉴别要点,提高病理诊断水平。方法 回顾性分析1例PC-iMCD患者临床资料,常规苏木素-伊红（HE）染色分析淋巴结组织结构及细胞形态,免疫组织化学染色及原位杂交分析免疫表型及EB病毒（EBV）感染状态,并结合文献分析讨论其与IgG4相关淋巴结病的鉴别诊断。结果 48岁女性患者,临床表现为口干、多饮、皮肤瘙痒伴全身多处淋巴结肿大。实验室检查血清IgG、IgA、IgM及IgE水平均升高,血清IgG4显著升高（14.7 g/L）,白介素- 6（IL-6）异常升高（150.84 pg/mL）。病理检查显示淋巴结生发中心萎缩,套区淋巴细胞呈“洋葱皮”样围绕生发中心排列,滤泡间区扩张,其内见大量成熟的浆细胞呈片状浸润,灶区见含铁血黄素沉积及血管增生;免疫组化染色显示IgG4阳性浆细胞数大于100/高倍视野,IgG4阳性细胞/IgG阳性细胞比值>40%,Kappa及Lambda轻链呈非限制性表达;EB病毒编码RNA原位杂交（EBER）阴性。结论 部分PC-iMCD与IgG4相关淋巴结病具有相似的组织病理学特征,单纯根据组织学及免疫表型难以将两者鉴别,正确诊断需结合IgG4相关病变诊断标准、排除性诊断标准、临床表现及实验室检查综合判断。

Objective By studying a rare case of the plasma cell type idiopathic multicentric Castleman disease（PC-iMCD）with histological characteristics similar to IgG4-related lymphadenopathy,the differential points of the two easily misdiagnosed diseases were summarized to improve the level of pathological diagnosis．Methods The clinical data of one patient with PC-iMCD were collected,the structure alteration and cell morphology were observed by hematoxylin-eosin（HE）stains．The immunophenotype of cells was marked by immunohistochemical staining and the infections status of EB virus was detected by in situ hybridization．Besides,the differential diagnosis between IgG4-RD and PC-iMCD were analyzed and discussed based on literature analysis．Results This article reported a 48-year-old female who was admitted to the hospital with dry mouth,polydipsia,skin itching and multiple lymphadenopathy．The levels of various classes of serum immunoglobulin were all increased,such as IgG,IgA,IgM and IgE．Specially,the serum IgG4 was also significantly increased（14.7 g/L）and interleukin 6（IL-6）was abnormally raised（150.84 pg/mL）．The pathological examination indicated that the lymph node germinal center was atrophied and mantle zones were expanded which were composed of concentric rings of lymphocytes in an “onion skinning” appearance．Besides,the interfollicular area was expanded in which mature plasma cells were infiltrated in sheet-like,hemosiderin was deposited as well as the vessels were proliferated．Immunohistochemistry showed that the number of IgG4-positive plasma cells was >100/HPF,IgG4/IgG-positive cells ratio was >40%,and Kappa and Lambda light chains were expressed unrestrictedly．In situ hybridization revealed that the expression of EBER was negative．Conclusions PC-iMCD and IgG4-related lymphadenopathy shares similar histopathological characteristics and it’s challenging to distinguish these two diseases by their histology and immunophenotype．In conclusion,the correct diagnosis should be combined with the diagnostic criteria,exclusion diagnostic criteria,clinical manifestations and related laboratory examinations．

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.

目的 基于影像组学方法,探讨多层螺旋CT（MSCT）四期增强扫描单一/不同期相及不同容积感兴趣区（VOI）的选择,在术前预测原发性肝细胞癌（HCC）微血管侵犯（MVI）中的价值。方法 回顾性收集88例经手术病理证实为HCC并行术前MSCT四期增强扫描的患者,其中包括47例MVI阳性患者和41例MVI阴性患者。在MSCT增强扫描的动脉早期、动脉晚期、门静脉期及延迟期图像中手动逐层勾画肿瘤ROI,获得瘤体容积感兴趣区VOI（Vt）,然后基于计算机自动膨胀算法将Vt外扩10 mm获得瘤体及瘤周VOI（Vt+Vp）。使用Pyradiomics软件分别从Vt和Vt+Vp中提取影像组学特征,随后采用15种特征选择方法和10种分类器构建150个预测模型,并通过十折交叉检验以验证模型的效能。使用准确度、敏感度、特异度、受试者工作特性曲线下面积（AUC）评估模型的效能,并比较性能最优的前三个预测模型。结果 MSCT四期增强扫描图像中预测HCC MVI状态的影像组学模型在门静脉期的表现优于其它期相及各期相的不同组合,其中最大的AUC值在Vt和Vt+Vp两种ROI中分别为0.768和0.782。此外,基于Vt+Vp的影像组学模型对MVI的预测效能优于基于Vt的影像组学模型,基于Vt+Vp性能最优的预测模型的AUC值、准确度、敏感度和特异度分别0.782、0.728、0.745和0.705。结论 采用影像组学方法术前无创性预测HCC MVI状态首选增强扫描的门静脉期,ROI首选瘤体联合瘤周10 mm区域。

Objective To investigate the value of single or different phases of contrast-enhanced multi-slice spiral CT（MSCT）in different volumetric regions of interest（ROI）to preoperatively predict the state of microvascular invasion in primary hepatocellular carcinoma（HCC）based on radiomics methods．Methods A total of 88 patients with HCC confirmed by surgical pathology who underwent preoperative MSCT quadruple-enhanced scan were retrospectively recruited,including 47 MVI-positive patients and 41 MVI-negative patients．The ROI was manually delineated slice-by-slice in the early arterial phase,late arterial phase,portal venous phase,and equilibrium phase of enhanced MSCT images to obtain the volume of tumor VOI（Vt）,and then Vt was expanded by 10 mm through the computer expansion algorithm automatically to obtain the volume of tumor and peritumor（Vt+Vp）．Pyradiomics software was used to extract radiomic features from Vt and Vt+Vp,followed by 150 discriminant models constructed with 150 feature selection methods and 10 classifiers,and then 10-fold cross-validation was used to evaluate the performance of these models．Using accuracy,sensitivity,specificity,area under the receiver operating characteristic curve（AUC）to assess model performance．The top three predictive models with the best performance were also compared．Results The radiomics model for predicting HCC MVI status in portal venous phase among quadruple-enhanced MSCT images outperformed other phases and different combinations of phases,achieving the highest AUC values of 0.768 and 0.782 in Vt and Vt+Vp respectively．In addition,the prediction performance of the radiomics model based on Vt+Vp was superior to models based on Vt．AUC value,accuracy,sensitivity,and specificity of the model with the best performance based on Vt+Vp were 0.782,0.728,0.745 and 0.705 respectively．Conclusions Radiomics models based on the portal venous phase of contrast-enhanced MSCT and tumor combined with the 10mm peritumoral area were more recommended to be employed to preoperative non-invasively predict the state of HCC MVI．

目的 对出生缺陷知信行健康教育模式效果进行策略分析与探讨,为进一步完善围产期保健服务及健康教育工作提供合理化建议。方法 采用《孕期妇女出生缺陷基本知识知晓率专题测评问卷》并结合实际情况设定有关信念、行为方面的7个问题,对2011年1月—2013年6月期间在中心孕妇学校参加健康教育活动课堂的孕妇进行调查,并应用决策树模型对中心现有的出生缺陷知信行健康教育模式效果进行探讨。结果 预防出生缺陷知识方面,对初高中及中专文化程度的孕期妇女加强出生缺陷预防知识健康教育的预期收益合计达到51.2%,可作为重点目标人群;预防出生缺陷信念方面,主要决定因素是户籍类型和年龄,加大对本地户籍及25岁以上流动妇女人群的健康教育,促进孕期妇女树立预防出生缺陷信念的预期收益累计达95.9%;预防出生缺陷行为方面,主要影响因素是产检医生的嘱咐,受到嘱咐的孕期妇女预期收益为62.3%。结论 为了达到出生缺陷防治目标与健康教育整体效果的提高,应将预防出生缺陷的健康教育工作重点放在初高中及中专文化程度人群,本地户籍孕期妇女和25岁以上流动性孕期妇女又是需要树立预防出生缺陷信念的重点人群,强化产检医生的嘱托义务是提高孕期妇女出生缺陷知信行健康教育模式效果的有力保证。

Objective To identify factors related to the knowledge, attitude and practice KAP of birth defects prevention among pregnant women, and provide reasonable suggestions to improve perinatal health services and health education. Methods Using cluster sampling method, 5500 participants from pregnant women school were enrolled. Information on “awareness of basic knowledge of birth defects” and 7 questions about attitudes and practice to participant actual situation was gathered through personal interviews using a structured questionnaire. The related factors were analyzed by classification tree model of SPSS 19.0. Results As to birth defects related knowledge, prospective return was more than 50% in the pregnant women with junior, senior high school and technical secondary school education level. As to birth defects prevention faith, the main determinant was census register types; prospective return of pregnant women who were local permanent residents and floating population aged ≥25 years was 95.9%. The main influencing factor of practice was prenatal doctor's education; prospected return was 62.3% after strengthening the health education of prenatal doctor. Conclusion In order to improve birth defects education effect, pregnant women who have junior, senior high school and technical secondary school education level and are local permanent residents or floating population aged ≥25 years should be key targeted population. Prenatal doctor's health education will be a powerful guarantee.