脾边缘带B细胞淋巴瘤的临床及病理组织学特征

目的：提高对脾边缘带淋巴瘤(SMZL)的认识和诊治水平。方法：对1例男性老年(75岁)SMZL患者的外周血、骨髓及脾脏标本，分别采用光镜、相差显微镜、透射电镜、免疫组织化学染色、RHG显带核型分析及PCR技术研究肿瘤细胞的生物学特征。结果：本例患者肿瘤细胞CD20、CD43、bcl—2表达阳性，肿瘤细胞呈结节状浸润脾脏白髓，致套区和边缘带完全被肿瘤细胞取代。骨髓细胞无异常核型。脾脏有单克隆IgH基因重排，骨髓和外周血未见异常淋巴细胞，无单克隆IgH基因重排。结论：对脾进行性肿大不伴浅表淋巴结肿大患者应疑为SMZL；单克隆IgH基因重排有助于SMZL的诊断，需排除滤泡中心淋巴瘤和套区淋巴瘤；脾切除治疗效果好。     

伴有绒毛细胞的脾边缘带B细胞淋巴瘤临床及实验室特征分析

结合文献对脾边缘带淋巴瘤（SMZL）疾病进行临床特点分析。本例患者肿瘤细胞为B淋巴细胞,部分伴有绒毛,脾脏病理示肿瘤细胞主要浸润脾脏白髓,呈结节状浸润,边缘带明显扩大,脾门淋巴结受累。病理细胞CD19、CD20、CD79a和CD22阳性表达,未见异常核型。认为脾大、外周血或骨髓淋巴细胞比例增高而无淋巴结肿大的患者应疑及SMZL,应注意与毛细胞白血病等疾病的鉴别,综合骨髓细胞形态、脾脏病理及免疫表型可作出临床诊断。     

以全血细胞减少为首发表现的脾边缘区淋巴瘤1例报告

脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)是指原发于脾脏的B淋巴细胞性非霍奇金淋巴瘤。SMZL的发病率低,国内以全血细胞减少为首发表现的SMZL报道较少。为提高临床医务人员对SMZL的认识,现将本院诊治的1例报道如下。1病例资料患者女性,76岁,以“腹胀、纳差1年,全血细胞减少3 d”为主诉于2016年6月23日于本院就诊。1年前曾于本院就诊,患者无明显诱因出现腹胀,伴有纳差,无腹痛、腹泻,无畏寒发热,无瘀斑瘀点,无咳嗽咳痰等不适,建议患者进一步检查明确原因,患者予以拒绝。     

眼结膜黏膜相关淋巴组织边缘区淋巴瘤临床病理分析

目的探讨眼结膜黏膜相关淋巴组织边缘区淋巴瘤临床病理特征、诊断、治疗及预后。方法回顾性分析了11例原发性眼结膜黏膜相关淋巴组织边缘区淋巴瘤患者的临床、病理资料并进行了随访。结果11例患者中男4例,女7例,发病年龄23～74岁,平均年龄为45岁。累及单侧结膜10例,双侧结膜1例,其他部位及系统未见淋巴瘤。病理形态:结膜固有层内弥漫性小-中等大小的淋巴样细胞及单核样细胞浸润,核形轻度不规则。免疫表型:弥漫性浸润细胞CD20(+)、CD3(-)、CD5(-)、CD10(-)、CD23(-)、Cyclin D1(-),5例异常表达CD43,ki67增殖指数为1%～10%。5例行Ig基因克隆性分析,均呈单克隆。6例单纯肿物完整切除,2例肿物完整切除加局部放疗,1例肿物部分切除加局部放疗,1例单纯活检,1例活检后局部注射化疗,肿物未消退,随后行肿物完整切除加局部放疗。随访时间3～122个月,截止随访日期,除2例失访,其他患者生存。其中行肿物单纯活检的患者长期带瘤生存,余8例未见复发。结论眼结膜黏膜相关淋巴组织边缘区淋巴瘤好发于中年女性,结膜见粉红色肿物为临床特征,镜下为小-中等大小的淋巴样细胞及单核样细胞弥漫性浸润,肿瘤细胞表达B淋巴细胞表型,增殖指数低,部分病例异常表达CD43,Ig基因克隆性分析有助于诊断,单纯肿物完整切除或术后辅以局部放疗均有较好的疗效,预后良好。     

晚期慢性淋巴细胞白血病伴脾大的脾切除治疗

慢性淋巴细胞白血病 (简称慢淋 )在我国发生率较低 ,在全部白血病中约占 4%。三分之二的患者在 60岁以上。我们曾对 3例晚期慢淋伴有显著脾大患者施行脾切除术 ,效果甚好 ,现报告如下。1　病例报告例 1　男 ,61岁。1 981年 1 1月因乏力在外院发现肝脾肿大 ,血常规异常 ,转来我院。当时白细胞为70× 1 0 9/L,血红蛋白为 1 1 7g/L,血小板为 63×1 0 9/L,经骨髓穿刺等检查确诊为慢淋 ,曾先后服用瘤可宁、CCNU等治疗。 1 990年 4月 1日因脾脏逐渐肿大、腹部胀满、明显贫血而再次入院。体检 :贫血貌 ,双侧锁骨下可触及数个黄豆大小淋巴结。心…     

以黄疸为表现并发自发性脾破裂的原发性脾淋巴瘤1例报告

正自发性脾破裂是临床罕见的非创伤性急症,常发生于病理性脾脏,主要因血液系统、炎症性、肿瘤性疾病导致,亦有相当一部分患者发生于无潜在疾病的脾脏~([1-2])。原发性脾淋巴瘤(primary lymphoma of spleen,PLS)并发自发性脾破裂既往仅有零星病例报道,现就1例以黄疸、自发性脾破裂就诊,行脾切除术后诊断为PLS的病例报告如下。     

肝脾γδT细胞淋巴瘤一例

患者女,22岁。因发热20余天伴全血减少,于2005年2月16日入我院血液科。患者无诱因出现发热,体温38～39℃,在当地医院检查发现脾肿大。全血细胞减少:WBC1.2×10~9/L、Hb 84 g/L、PLT 56×10~9/L。异型淋巴细胞0.11。拟病毒感染,伤寒待排。用抗生素、抗病毒治疗无效。入院体检:T 36.3℃、P 82次/min、R 20次/min、BP 12/8kPa(1 kPa=7.5 mm Hg)。神清,轻度贫血貌,皮肤黏膜无出血,无皮疹,浅表淋巴结无肿大。心肺无异常,肝肋下未及,脾肋下2 cm。诊断:恶性组织细胞增生症?淋巴瘤?传染性单核细胞增多症?     

外周T细胞淋巴瘤临床分析

2001年颁布的WHO淋巴肿瘤新分类中认为外周T细胞淋巴瘤-非特指型（PTCL-U）是来源于胸腺后T淋巴细胞的一大类恶性肿瘤。这是一类排除性疾病，它包括除特指的外周T细胞以外的所有不能分型的外周T细胞淋巴瘤，即诊断前需除外T／NK细胞淋巴瘤、淋巴母细胞淋巴瘤、间变大细胞淋巴瘤等。本病有明显的区域分布，在欧美国家发病率较低，约占非霍奇金淋巴瘤（NHL）7％，而在我国及亚洲国家发病率较高，约占T细胞NHL的50％，占NHL的15％。目前，对PTCL-U的临床表现、病理形态、免疫学及细胞遗传学的研究有限，认识不足，近年来受到国内外学者的关注。现将我院2001年7月至2004年6月的23例PTCL—U进行回顾性分析，探讨其临床表现、诊断、治疗及预后。     

Splenectomy with chemotherapy vs surgery alone as initial treatment for splenic marginal zone lymphoma

AIM： To evaluate the clinical characteristics of splenic marginal-zone lymphoma （SMZL） following antigen expression and the influence of therapeutic approaches on clinical outcome and overall survival （OS）.
METHODS： A total of 30 patients with typical histological and immunohistochemical SMZL patterns were examined. Splenectomy plus chemotherapy was applied in 20 patients, while splenectomy as a single treatment-option was performed in 10 patients. Prognostic factor and overall survival rate were analyzed.
RESULTS： Complete remission （CR） was achieved in 20 （66.7%）, partial remission （PR） in seven （23.3%）, and lethal outcome due to disease progression occurred in three （10.0%） patients. Median survival of patients with a splenectomy was 93.0 mo and for patients with splenectomy plus chemotherapy it was 207.5 mo （Log rank = 0.056, P 〉 0.05）. Time from onset of first symptoms to the beginning of the treatment （mean 9.4 too） was influenced by spleen dimensions, as measured by computerized tomography and ultra-sound （t = 2.558, P = 0.018）. Strong positivity （＋＋＋） of CD20 antigen expression in splenic tissue had a positive influence on OS （Log rank = 5.244, P 〈 0.05）. The analysis of factors interfering with survival （by the Kaplan-Meier method） revealed that gender, general symptoms, clinical stage, and spleen infiltration type （nodular vs diffuse） had no significant （P 〉 0.05） effects on the OS. The expression of other antigens （immunohistochemistry） also had no effect on survival-rate, as measured by a χ^2 test （P 〉 0.05）.
CONCLUSION： Initial splenectomy combined with chemotherapy has been shown to be beneficial due to its advanced remission rate/duration; however, a larger controlled clinical study is required to confirm our findings.     

Laparoscopic splenectomy for treatment of splenic marginal zone lymphoma

AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.     

Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination

The simultaneous occurrence of Hodgkin lymphoma with a variety of B-cell Non-Hodgkin lymphomas (composite lymphoma) has been described. We report the first case of composite Hodgkin lymphoma and splenic marginal zone lymphoma occurring simultaneously in the same lymph node of a 64-year-old man who presented with cervical and axillary lymphadenopathy and massive splenomegaly. He had a peripheral blood lymphocytosis and a bone marrow infiltrated by small lymphocytes. However, cervical lymph node biopsy showed classic Hodgkin lymphoma. His splenomegaly showed only a partial response to six cycles of ABVD chemotherapy so he underwent splenectomy with biopsy of remaining nodes. Histology of the spleen and nodes showed splenic marginal zone lymphoma. Review of the original biopsy confirmed the presence of both diseases in the original lymph node.     

Primary nodal marginal zone B-cell lymphoma: clinical features and prognostic assessment of a rare disease

This study defined the clinical features and assessed the prognosis of 47 patients (17 males, 30 females, median age 63 years) with primary nodal marginal zone B-cell lymphoma. Forty-five per cent had stage IV disease. Hepatitis C virus serology was positive in 24%. According to the Follicular Lymphoma International Prognostic Index (FLIPI), 33% were classified as low-risk, 34% as intermediate-risk, and 33% as high-risk. The 5-year overall survival (OS) was 69%. In univariate analysis worse OS was associated with: FLIPI (P = 0.02), age > 60 years (P = 0.05) and raised lactate dehydrogenase (P = 0.05). In multivariate analysis, only FLIPI predicted a worse OS (P = 0.02).     

Splenic marginal zone lymphoma associated with hepatitis B virus infection,remission after viral treatment,and splenectomy: A case report and review of the literature

We report the case of a patient diagnosed with a splenic marginal zone lymphoma with a simultaneous finding of hepatitis B virus infection, who responded to antiviral treatment and splenectomy. We highlighted this association described in the literature and its possible causal role, as well as the available therapeutic choices.     

Stereotyped patterns of B-cell receptor in splenic marginal zone lymphoma

Antigen stimulation may be important for splenic marginal zone lymphoma pathogenesis. To address this hypothesis, the occurrence of stereotyped B-cell receptors was investigated in 133 SMZL (26 HCV+) compared with 4,414 HCDR3 sequences from public databases. Sixteen SMZL (12%) showed stereotyped BCR; 7 of 86 (8%) SMZL sequences retrieved from public databases also belonged to stereotyped HCDR3 subsets. Three categories of subsets were identified: i) "SMZL-specific subsets" (n=5), composed only of 12 SMZL (9 HCV-from our series); ii) "Non-Hodgkin’s lymphoma-like subsets" (n=5), comprising 5 SMZL (4 from our series) clustering with other indolent lymphomas; iii) “CLL-like subsets” (n=6), comprising 6 SMZL (3 from our series) that belonged to known CLL subsets (n=4) or clustered with public CLL sequences. Immunoglobulin 3D modeling of 3 subsets revealed similarities in antigen binding regions not limited to HCDR3. Overall, data suggest that the pathogenesis of splenic marginal zone lymphoma may involve also HCV-unrelated epitopes or an antigenic trigger common to other indolent lymphomas.