膀胱原发性弥漫大B细胞淋巴瘤1例

<正>患者女性,77岁,因尿频、尿急40余天伴肉眼血尿10余天入院。患者于2017年2月16日在当地县医院就诊,并行活检,病理诊断:考虑为膀胱浸润性尿路上皮癌,未行免疫组化检查。于2017年2月底转入我院,CT示膀胱内多发占位性病变,考虑为恶性肿瘤。膀胱镜检查示:膀胱右侧壁可见一直径6. 5 cm的菜花样肿物,左后侧壁可见一直径3 cm的菜花样肿物,均存在活动性出血。行经尿道膀胱肿瘤电切术,切除组织送病理检查。     

原发性皮肤B细胞淋巴瘤研究进展

皮肤的Ｂ细胞淋巴瘤曾被认为是淋巴造血系统结内淋巴瘤的一个结外表现 ,且提示预后差。目前认为原发性皮肤Ｂ细胞淋巴瘤 (ｐｒｉｍａｒｙｃｕｔａｎｅｏｕｓＢｃｅｌｌｌｙｍｐｈｏｍａｓ ,ＰＣＢＣＬｓ)是一组细胞形态学高度异质 ,临床表现独特 ,起源于皮肤并常常只局限于皮肤的独立疾病[1,2 ] 。大部分患者的临床表现是非进展性的 ,与同类结内淋巴瘤相比 ,预后较好。近年来 ,虽然对该病的认识在不断提高 ,但对其分类仍存在着分歧。我们对目前ＰＣＢＣＬｓ的分类方案、主要临床类型、病理特点及其与皮肤的淋巴组织增生性疾病的鉴别进行了回…     

皮肤血管内大B细胞淋巴瘤1例

患者女性,65岁.右下肢皮肤斑块伴破溃,遂于2010年6月做皮肤活检,诊断为血管内大B细胞淋巴瘤(intravascular large B cell lymphoma,IVLBCL).实验室检查:WBC 5.85×109/L,Hb 122 g/L,Plt 88×109/L,分叶核细胞0.71,杆状核细胞0.01,淋巴细胞0.28,变异淋巴细胞0.02,晚幼红细胞3个/100个有核细胞.肝功能:总胆红素、直接和间接胆红素正常,谷丙转氨酶(ALT)80 IU/L,天冬氨酸转氨酶(AST)82 IU /L,总蛋白58.4 g/L,白蛋白26.6 g/L,球蛋白27.7 g/L,碱性磷酸酶225 IU /L,谷氨酰转肽酶(GGT)158 IU/L,乳酸脱氢酶(LDH)464 IU/L,羟丁酸脱氨酶(HBDH)387IU/L.     

弥漫大B细胞淋巴瘤基因表达谱的研究进展

弥漫大B细胞淋巴瘤（DLBCL）是最常见的一类淋巴瘤,占非霍奇金淋巴瘤的30％左右。DLBCL的临床表现、形态学、免疫表型及遗传学特征极具异质性,越来越多的证据表明其可能并不是一个真正独立的病种。最新的WHO淋巴造血组织肿瘤分类列出了DLBCL的一些形态学亚型,但这种形态学分型不仅与预后的关系尚存在争议,而且诊断的可重复性差,所以实际应用价值不大。大多数DLBCL患者经过化学治疗后可以获得缓解,但半数患者仍在短期内复发并死亡。国际预后指数（international prognostic index,IPI）是通过年龄、功能状态、血清乳酸脱氢酶水平、累及结外部位的数量以及肿瘤临床分期等5个临床特征对患者进行评分,具有相当的预后判断价值。     

前列腺血管内弥漫大B细胞淋巴瘤1例

患者,男,78岁.因"脑梗塞、高血压"于2007年8月29日入院.在内科治疗6天后脑梗塞症状好转,出现急性尿潴留症状,询问病史,以往也有尿频、尿淋漓不禁的症状,B超提示"前列腺肥大,光点分别欠均匀,建议进一步检查",转入外科进行手术治疗.手术前患者血常规、肝功能、全胸片正常,全身浅表淋巴结无肿大.手术所见:膀胱内小梁小室形成,未见新生物,前列腺肥大,突人膀胱,常规电切前列腺组织至包膜.     

外耳道原发性皮肤间变性大细胞淋巴瘤1例

患者男性,80岁.因右耳道流水1个月余,保守治疗无效,后来本院就诊.当地医院CT检查示:右侧外耳道软组织影.本院门诊拟"右侧外耳道肿物"收入院.体检:右侧外耳道肿胀、充血,黏脓性分泌物,鼓膜窥不见.全身浅表淋巴结及肝脾无肿大.病程中患者无发热,血、尿、粪常规和肝、肾功能均正常.遂行右侧乳突根治术+外耳道肿物切除术.术中见外耳道全部被新生物占据,新生物穿破外耳道后壁骨膜侵犯骨质.     

肾脏原发性弥漫性大B细胞淋巴瘤1例

患者女,61岁,左腰痛4个月余来院就诊.CT及增强造影显示:左肾见占位性病变.临床拟"肾癌"收入院.术中见左肾体积增大,与周围粘连,腹主动脉旁有肿大淋巴结,行"左肾切除术",腹主动脉旁淋巴结清扫.标本经10%福尔马林液固定,石蜡包埋,3 μm厚连续切片,作常规HE及免疫组化标记.免疫组化试剂由福州迈新公司提供.     

p63阳性的弥漫大B细胞淋巴瘤1例并文献复习

弥漫大B细胞淋巴瘤是最常见的非霍奇金淋巴瘤,p63在弥漫大B细胞淋巴瘤中可表达,因此在临床上易被误诊为p63阳性的上皮源性恶性肿瘤,尤其在淋巴结发生转移时.本研究报道1例以呼吸道症状为首发症状的弥漫大B细胞淋巴瘤患者,临床高度怀疑肺恶性肿瘤并淋巴结转移.行右腋窝淋巴结穿刺活检病理诊断,结果显示p63阳性,提示为鳞状细胞...     

EBV阳性弥漫大B细胞淋巴瘤18例临床病理分析

目的观察EBV阳性弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL)的临床病理特点并探索可能的预后相关因素。方法回顾性分析2012年～2017年间福建医科大学附属协和医院诊断的18例EBV阳性DLBCL病例临床特点、病理形态、免疫表型及EBER原位杂交等相关资料,并随访患者,采用Kaplan-Meier法进行生存分析。结果EBV阳性DLBCL仅占EBV感染状态明确DLBCL病例的3. 7%(18/483);发病年龄33～79岁,中位发病年龄69岁;男女比为2. 6∶1; 50岁以下的患者仅4例(22%,4/18);发病部位以淋巴结病变为主;临床上多数患者血常规表现为贫血、白细胞减少或血小板减低,血乳酸脱氢酶水平明显增加; 9例血清EBV DNA载量明显升高(64. 3%,9/14);肿瘤细胞形态以多形性为主(88. 9%,16/18)。免疫表型非生发中心起源型(non-germinal center origin subtype,non-GCB型)稍多(55. 5%,10/18),多数病例表达CD30(62. 5%,10/16);患者随访2～47个月,8例死亡,7例存活,3例失访,中位生存时间为16个月;发病部位的不同与患者生存时间的长短有一定的关系(P=0. 022)。结论 EBV阳性DLBCL是一类以老年男性为主、淋巴结病变多见的高侵袭性淋巴瘤,病理以多形性型及non-GCB免疫表型为主,总体病死率高,仅发生淋巴结病变的患者预后相对更差。     

Three cases of diffuse large B-cell lymphoma presenting as primary splenic lymphoma

Primary splenic lymphoma (PSL) is often defined as generalized lymphoma with splenic involvement as the dominant feature. It is a rare disease that comprises approximately 1% of all malignant lymphomas. We investigated three cases of non-Hodgkin's splenic lymphoma that had different clinical features on presentation. The patients' survival times from diagnosis ranged from 59 to 143 months, without evidence of relapse after splenectomy and chemotherapy, with or without radiotherapy. This data suggest that PSL is potentially curable. Further studies are needed to evaluate the impact that different treatment modalities without splenectomy have on patient survival.     

Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma

Primary CNS diffuse large B-cell lymphoma (CNS DLBCL) is confined to the CNS, and constitutes a distinct entity. In the present study a series of 40 Japanese patients with CNS DLBCL who presented with neurological, but not systemic symptoms, was reviewed. Median survival was 18.7 months. CD5, CD10, Bcl-6, MUM-1, and Bcl-2 were positive in 30%, 10%, 84%, 100%, and 93% of patients, respectively. All CD10-negative patients had non-germinal center B-cell type. There was no significant difference in survival among the immunophenotypic subgroups. CNS DLBCL appeared to be homogenous as a group, which prompted the comparison with another distinct extranodal entity, intravascular large B-cell lymphoma (IVLBCL) in Japanese patients. CNS DLBCL patients did not differ in age, sex, or immunophenotype, including CD5 positivity, from IVLBCL patients, but were significantly less likely to have poor prognostic parameters than IVLBCL patients: the international prognostic index score was low or low–intermediate in 86% of CNS DLBCL patients and high or high–intermediate in 98% of IVLBCL patients. Notably, despite this difference, their survival curves almost overlapped. The present study highlights the issue of clinical distinctiveness of aggressive extranodal lymphomas, the peculiar migration and localization of which should be further clarified.     

One patient with double lymphomas: simultaneous gastric MALT lymphoma and ileal diffuse large B-cell lymphoma

Multiple different lymphomas in a single person are very rare. The author herein reports the case of a 69- year-old Japanese woman with double gastrointestinal lymphoma. The patient presented with epigastralgia. Endoscopic examination revealed erosions and elevation of the gastric body and a large ulcerated tumor of the terminal ileum. Biopsies were obtained from these lesions. The gastric lesion was MALT lymphoma with monocytoid B-cell proliferation and lymphoepithelial lesions. Light chain restriction was present. Helicobacter pylori were present on Giemsa stain. The gastric lesions did not regress despite of therapy, which were confirmed by follow-up biopsy. The ileal lesion was obvious diffuse large B-cell lymphoma. The lesion regressed by chemotherapy. The patient is now alive 3 years after the first presentation.     

原发心脏弥漫大B细胞淋巴瘤5例临床病理分析

目的 探讨原发心脏弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)的临床病理特点、诊断、治疗及预后.方法 回顾性分析5例原发心脏DLBCL的临床病理资料,分别行HE染色、免疫组化EnVision两步法染色,应用原位杂交以及荧光原位杂交(fluorescence in situ...     

Primary cutaneous B-cell lymphoma in Japanese patients

Primary cutaneous B-cell lymphoma (PCBCL) is a rare group of lymphoproliferative disorders. There have been few reports of Japanese patients with PCBCL, so the present study investigated the clinicopathological and immunological features and Bcl-2 gene rearrangement and protein expression in 28 Japanese patients with PCBCL. According to the Revised European-American Lymphoma (REAL) classification, there were 25 diffuse large B-cell lymphomas (DLBCL), one Burkitt type lymphoma, one lymphoblastic lymphoma and one marginal zone cell lymphoma. Of the 25 DLBCL, 17 were in males and eight in females, with an average age of 69.4 years. Follow-up data were available in 19 cases of DLBCL of which seven died and 12 were alive. The overall 5-year survival rate was 61%. Cases of DLBCL involving the legs were found to have poorer clinical outcomes; two of four cases with leg lesions died, with a mean survival of 13 months. Of 14 cases with non-leg lesions, four died, and the mean survival was 38.9 months. Only one case of Burkitt type lymphoma was CD10 positive. Bcl-2 rearrangement was not observed in 13 cases studied by polymerase chain reaction. Bcl-2 expression was observed in nine of 13 cases studied. All five cases with leg lesions exhibited Bcl-2 expression, but four of six cases with non-leg lesions also expressed the protein. These results show that DLBCL is the most frequent subtype of PCBCL in Japanese patients and that the prognosis of Japanese patients with DLBCL is worse than that of reported European cases. The study also found that PCBCL was frequently associated with Bcl-2 expression, which was not site-confined, and that there was no evidence for a follicular center origin of PCBCL.     

Composite primary breast diffuse large B-cell lymphoma and T lymphoblastic leukemia/lymphoma: report of a case and review of literature

We reported a rare case of composite diffuse large B-cell lymphoma and T lymphoblastic leukemia/lymphoma (T-LBL) in a 46-year-old woman with progressive enlargement of the breast lump. The patient initially sought care at a local hospital with a single left breast lump without any other physical examination findings. Histopathological analysis of which revealed a diffuse infiltration of tumor cells that were rich in cytoplasm with vesicular chromatin and prominent nucleoli. Further analysis of immunohistochemistry showed a cluster of neoplastic cells which express B-cell markers: CD19, CD20 (weak), CD79a, PAX5 and BCL-2, but negative for T-cell markers such as CD2, CD3, CD5 and CD7. PET-CT showed evidence of lymphadenopathy and splenomegaly, which may indicate lymphoma infiltration. Then a biopsy of bone marrow showed typical features of T-LBL. The aberrant terminal deoxynucleotidyl transferase (TDT) and cCD3 positive T-cell population that lack surface CD10 and CD19 were identified by flow cytometric immunophenotyping. Polymerase chain reaction analysis of the T-cell receptor gamma gene and IgH gene revealed a clonal rearrangement and confirming T-cell clonality. Fluorescence in-situ hybridization (FISH) revealed a deletion of the P53 gene in these T-neoplastic cells may indicate a bad outcome of such disease. Neither the large B-cells nor T-cells were positive for Epstein-Barr virus encoded RNA.     

Development of angioimmunoblastic T-cell lymphoma after treatment of diffuse large B-cell lymphoma: a case report and review of literature

Cases of diffuse large B-cell lymphoma (DLBCL) arising after the initial diagnosis of angioimmunoblastic T-cell lymphoma (AITL) and DLBCL synchronous with AITL have been reported. To date, there is no report on the subsequent development of AITL in patients with DLBCL. Here we presented a rare case of AITL developing six months after the initial diagnosis of DLBCL. In order to investigate the clinical and molecular features of patients with AITL and DLBCL, we also reviewed the literature on AITL patients developing DLBCL, and patients with composite AITL and DLBCL.     

Prognostic significance of activation and differentiation antigen expression in B-cell non-Hodgkin's lymphoma

Immunophenotyping shows heterogeneity of expression of activation and differentiation antigens in B-cell non-Hodgkin's lymphoma (NHL). To investigate whether antigen expression correlates with clinical behaviour we have studied the clinical presentation and follow-up of a series of 111 B-cell lymphomas previously phenotyped for a panel of antigens including CD groups 5, 9, 10, 21, 23, 25, 30, 38, 4F2 antigen, and transferrin receptor. CD antigens 5, 10, and 23 were expressed significantly more often by low grade lymphomas whereas CD38, 4F2 antigen, and transferrin receptor were more often expressed by high grade lymphomas. There was a significant correlation with survival and age, stage at presentation, histological grade, and expression of 4F2 antigen and transferrin receptor but not with the other antigens studied. 4F2 antigen and transferrin receptor may identify a poor prognostic group of cases in low grade lymphoma but we conclude that phenotyping B-cell NHL for many of the antigens expressed at various stages of B-cell differentiation and activation does not provide clinically useful information in addition to that obtained from standard histological classifications.