ObjectiveTo retrospectively analyze the reasons for misdiagnosis of haematolymphoid neoplasms and provide experience for improving the diagnostic level in China. MethodsA retrospective analysis was performed on 2291 cases of haematolymphoid diseases evaluated by the Department of Pathology of our hospital from 1 July 2019 to 30 June 2021. All 2291 cases were reviewed by two hematopathologist experts and classified according to the 2017 revised WHO classification criteria, supplemented immunohistochemistry (IHC), molecular biology and genetic information as needed. The diagnostic discordance between primary and expert review was evaluated. The possible causes of the diagnostic discrepancies were analyzed for each step involved in the procedure of diagnosis. ResultsIn total, 912 cases did not conform to the expert diagnoses among all the 2291 cases, with a total misdiagnosis rate of 39.8%. Among them, misdiagnosis between benign and malignant lesions accounted for 24.3% (222/912), misdiagnosis between haematolymphoid neoplasms and non-haematolymphoid neoplasms accounted for 3.3% (30/912), misdiagnosis among lineages accounted for 9.3% (85/912), misclassification in lymphoma subtypes accounted for 60.8% (554/912), and other misdiagnoses among benign lesions accounted for 2.3% (21/912) of cases, among which misclassification of lymphoma subtypes was the most common. ConclusionThe accurate diagnosis of haematolymphoid neoplasms is challenging, involving various types of misdiagnosis and complicated causes, however, it is important for precise treatment. Through this analysis, we aimed to highlight the importance of accurate diagnosis, avoid diagnostic pitfalls and to improve the diagnostic level in our country.

患者, 女, 25岁。患者于2022年9月发现颈部左侧有一约1.3 cm×0.8 cm大小肿块, 质硬, 无触痛, 活动性差, 就诊于当地医院, 行颈部超声示:左颈部Ⅱ区可见3.8 cm×2.8 cm×1.6 cm实性肿物, 予口服及静脉应用抗生素后未见好转。患者10月12日行超声引导下肿物穿刺, 术后病理示:(颈部左侧肿物)淋巴组织增生。患者2022年10月就诊于解放军总医院第一医学中心, 行PET/CT检查, 结果示:颈部左侧血管旁多发增大淋巴结, FDG摄取异常增高, 最大标准摄取值(SUVmax)=23.1, 病变边界不清, 与相邻左侧颌下腺、左侧胸锁乳突肌分界不清, PET/CT融合图显示大者约3.4 cm×2.7 cm×3.6 cm, 病变密度尚均匀, CT值约60 Hu。双侧锁骨区、双侧腋窝、双侧腹股沟区未见明显肿大淋巴结。患者2022年11月行颈部左侧肿物切除术, 术中送检(左颈Ⅱ区)淋巴结5枚, 大者1.0 cm×0.7 cm×0.5 cm, 小者0.2 cm×0.2 cm×0.2 cm。术后送检(左颈Ⅱ区)淋巴结3枚, 大者4.5 cm×4.0 cm×1.5 cm, 部分被膜破损, 切面灰红色及灰白色, 质地中等;小者0.3 cm×0.2 cm×0.2 cm, 质地中等。病理示:(左颈Ⅱ区)淋巴结B细胞源性淋巴瘤, 考虑血管内大B细胞淋巴瘤可能性大。

目的:提高对伴低Ki-67表达的肝脾T细胞淋巴瘤的认识。方法:回顾性分析北京高博博仁医院2020年会诊的2例肝脾T细胞淋巴瘤患者的临床及病理资料，并复习肝脾T细胞淋巴瘤相关文献。结果:例1脾脏及骨髓活组织检查标本及例2肝脏、脾脏及骨髓活组织检查标本见到形态较单一T淋巴细胞增生，可见抗原丢失，增殖活性低，结合细胞形态学及免疫组织化学结果，最终均诊断为肝脾T细胞淋巴瘤，伴低增殖活性。后续均行异基因造血干细胞移植，随访至2023年5月患者一般情况良好。结论:伴低Ki-67表达的肝脾T细胞淋巴瘤较少见，早期造血干细胞移植可能获得较好的临床预后。

Objective: To explore the feasibility of predicting TP53 mutation risk by immunohistochemical staining (IHC) pattern of P53 in Chinese diffuse large B-cell lymphoma (DLBCL) and its correlation with a prognostic difference. Methods: Between January 2021 and December 2021, 51 DLBCL cases at Beijing Boren Hospital were gathered. These cases had both IHC and next-generation sequencing (NGS) results. IHC classified the P53 protein expression pattern into a loss (<1% ) , diffuse (>80% ) , and heterogeneous (1% -80% ) . The sensitivity and specificity of the predicting TP53 mutation by IHC were assessed by comparing the results of the NGS, and the TP53 high mutation risk group included both loss and diffuse expression of P53. From June 2016 to September 2019, Peking University Cancer Hospital collected 131 DLBCL cases with thorough clinicopathological and follow-up data. From their tumor blocks, tissue microarray blocks were made for IHC evaluation of P53 expression pattern, and prognosis effect of P53 studies. Results: Among 51 cases with both IHC and NGS results, 23 cases were classified as TP53 high mutation risk (7 cases loss and 16 cases diffuse) , 22/23 cases were proved with mutated TP53 by NGS. Only 1 of the 28 cases classified as TP53 low mutation risk was proved with mutated TP53 by NGS. IHC had a sensitivity and specificity of 95.7% and 96.4% for predicting TP53 mutation. NGS identified a total of 26 TP53 mutations with a mutation frequency of 61.57% (13.41% -86.25% ) . In the diffuse group, 16 missense mutations and 2 splice mutations were detected; 6 truncating mutations and 1 splice mutation were detected in the loss group; 1 truncating mutation was detected in the heterogeneous group. Multivariate analysis demonstrated that TP53 cases with high mutation risk have impartial adverse significance for the 131 patients included in survival analysis (HR=2.612, 95% CI 1.145-5.956, P=0.022) . Conclusion: IHC of P53 exhibiting loss (<1% ) or diffuse (>80% ) pattern indicated TP53 high mutation risk, IHC can predict TP53 mutation with high specificity and sensitivity. TP53 high mutation risk is an independent predictor for adverse survival.

Objective:To explore the key points of the pathological and differential diagnoses of extra-medullary masses of hematopoietic cell tumors of ambiguous lineage, and to discuss the possible solutions.Methods:Five hematopoietic cell tumors of ambiguous lineage cases were collected, including myeloid sarcoma, mixed phenotype acute leukemia, B/myeloid, T-lymphoblastic lymphoma combined with acute myeloid leukemia, acute undifferentiated leukemia with cutaneous MPDCP and early T-precursor cell acute lymphoblastic leukemia. The data including morphology, immunostaining, and flow cytometry analysis were collected, and we explored the problems and differential diagnosis in the diagnosis of hematopoietic cell tumors of ambiguous lineage.Results:The five cases showed that the accurate pathological diagnosis and classification of hematopoietic cell tumors of ambiguous lineage should be based on lineage-specific antigens. Moreover, tumor cells have the potential of multi-directional differentiation. In different sites or different periods, the differentiation of tumor cells may be different. Biopsy and detection of all related markers should be performed for the initial diagnosis, and the detection should be repeated when the condition of the patient changes. Combined application of multi-techniques, including morphology and flow cytometry analysis, is recommend for the diagnosis of hematopoietic cell tumors of ambiguous lineage, since the conventional morphology and immunophenotyping methods are limited.Conclusion:Hematopoietic cell tumors of ambiguous lineage are derived from hematopoietic stem cells with a potential of multi-differentiation. The differentiation of tumor cells is variable. We need to integrate cell morphology, flow cytometry, pathology, clinical data, and molecular genetics to make a comprehensive diagnosis.

目的 观察鳖甲生血丸治疗原发骨髓纤维化的临床疗效及不良反应.方法 收集原发性骨髓纤维化患者120例,给予鳖甲生血丸8 g,每日2～3次,口服,共治疗3个月.观察血常规,肝、脾情况,骨髓检查及不良反应. 结果 120例患者好转39例,进步47例,无效34例,总有效率71.67％.30例血红蛋白、白细胞、血小板减低患者治疗后血红蛋白、白细胞显著升高(P＜0.05).白细胞、血小板计数增高的患者,治疗后血小板计数明显降低(P＜0.05).114例脾肿大患者脾大情况治疗后与治疗前比较差异有统计学意义(P＜0.05);81例肝肿大患者肝大情况治疗后与治疗前比较差异无统计学意义(P＞0.05).骨髓病理活检显示治疗后骨髓纤维化程度有明显改善(P＜0.05).120例患者中11例出现少量皮肤出血点,7例有消化道不良反应,减量后消失.结论 鳖甲生血丸治疗原发性骨髓纤维化有较好的近期疗效,无严重不良反应.