胃肠道原发B细胞性淋巴瘤的病理学特征及播散机制

目的研究胃肠道原发B细胞性淋巴瘤的病理学特征及淋巴细胞归巢受体CD44在其播散中的作用。方法对199例胃肠道原发B细胞性淋巴瘤进行病理组织学及免疫组化研究。结果胃肠道原发B细胞性淋巴瘤以DL(70.9%)发病率最高,MALT淋巴瘤(9.5%)次之。LN1染色在MALT淋巴瘤、MZC均为核周点状阳性。1例MLP肿瘤细胞LN1染色特性及形态学特征与MALT淋巴瘤相同。199例中单克隆性cIg检出率为49.7%,在MALT淋巴瘤为100%。临床Ⅲ、Ⅳ期组的CD44阳性率明显高于Ⅰ、Ⅱ期组(P<0.001);在全身弥漫播散病例CD44阳性率高于MALT内播散者。结论MALT淋巴瘤来源于MZC,其惰性生物学行为与细胞来源有关。MLP除可为多中心起源的套细胞来源外,也可是MALT淋巴瘤晚期扩散的结果。cIg检测有助于胃肠道淋巴组织良恶性疾病的鉴别诊断。CD44表达与淋巴瘤的血道播散密切相关。     

15例原发性非霍奇金脾淋巴瘤的临床、病理特征

目的：研究原发性非霍奇金脾淋巴瘤（脾ＮＨＬ）的临床和病理特征与手术治疗的效果。方法：１９６４年－１９９８年本院获确诊并治疗１５例原发性非霍奇金脾淋巴瘤，并作术后随访。结果：ＮＨＬ的主要临床表现为左上腹痛或不适，脾肿大，贫血和发热，Ｂ超显示脾内多发性占位。Ａｈｍａｎｎ’ｓ分期（１４例）：Ⅰ期３例，Ⅱ期４例，Ⅲ期７例，脾切除及脾切除联合腹腔其他脏器切除１３例。术后均予化疗，其中２例配合放疗，术后生存３     

类胰蛋白酶在冠心病患者尸检心脏组织中的表达

目的探讨类胰蛋白酶(tryptase)与冠心病病情及与冠状动脉病变程度的关系。方法从本教研室2008～2011年尸检档案中挑选病例及心脏标本共70例。分为四组:A组:冠心病猝死(SCD,20例);B组:冠心病非猝死者(CHD,20例);C组:过敏性猝死(10例);D组:阴性对照(无明显动脉粥样硬化病变的死者,20例)。应用免疫组化染色(SP法)和图像定量分析法,观察每例左心室缺血心肌的类胰蛋白酶染色情况和光密度。结果 SCD组、CHD组、过敏性猝死组光密度值均高于阴性对照组,各组间比较均有显著差异(P<0.01)。结论缺血心肌中类胰蛋白酶的表达增加与SCD的发生密切相关,为SCD的诊断和临床对冠心病防治的研究提供新的途径。     

42 例原发性胃淋巴瘤的临床特点分析

目的：探讨原发性胃淋巴瘤（PGL）的临床特点,以期提高该病的诊治水平。方法：回顾性分析湖北医药学院附属太和医院2003年01月至2013年01月收治的42例原发性胃淋巴瘤患者的临床病理资料。结果：患者多以消化道症状为主要表现,腹痛最常见,占83．3％,其次为恶心、呕吐、黑便和腹部包块。胃镜诊断阳性率为46．1％,高于钡餐及cT,超声胃镜可以协助诊断。DLBCL是其最常见的病理类型。IE期23例、ⅡE期11例、ⅢE期5例、ⅣE期3例。MALToma的预后与DLBCL无显著差异。LDH升高者预后较正常水平者差;IE期、ⅡE期患者预后较ⅢE期、ⅣE期好。结论：PGL临床表现以消化道症状为主,胃镜检查有利于诊断,治疗手段包括手术、化疗及放疗,选用CHOP化疗方案的患者联合美罗华预后较仅行CHOP方案好。     

Clinical features of extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue

We newly diagnosed 131 patients with extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue lymphoma between 1998 and 2010. We retrospectively studied 124 patients for whom complete clinical data were available at presentation and who had minimally undergone tumour staging by physical examination, computed tomography (CT), bone marrow aspiration, and biopsy. A slight female predominance (men, 58; women, 66) was observed in the study population; the median age was 67 years. The primary locations at presentation were the stomach (38%), orbita (20%), lung (12%), intestinal tract (8%), thyroid gland (6%), others (14%), and unknown (2%). Seventy per cent of patients had localized disease. Of the 124 patients, 14 (11%) had lymph node involvement, and 5 (4%) had bone marrow involvement. Five (4%) patients had both lung and gastric involvement. The 5‐year overall survival rate for the 124 patients was 96.1%. The overall vital prognosis was excellent. Moreover, gastro‐intestinal fiberscopic examination is essential, especially in cases with lung involvement at presentation. Copyright © 2012 John Wiley & Sons, Ltd.     

Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases

We analysed the clinical and pathologic features of 42 patients with immunologically confirmed peripheral T-cell lymphoma. The median age was 60 years and the male to female ratio was 1:1. A prior lymphoproliferative or autoimmune disorder was present in 14 per cent of the patients. Signs of advanced disease were usually present from the onset, such as B symptoms (55 per cent), generalized lymphadenopathy (57 per cent), stage III/IV disease (62 per cent), and elevated levels of serum lactate dehydrogenase (68 per cent). Primary extranodal disease (14 per cent), hepatomegaly (12 per cent), splenomegaly (12 per cent), lung/pleural involvement (12 per cent), skin involvement (21 per cent), and bone marrow involvement (28 per cent) were uncommon. Lymphocytopenia was present in 64 per cent of the patients, and none of nine patients tested were serologically positive for human T-cell leukemia/lymphoma virus (HTLV-I) infection. Among 38 patients receiving combination chemotherapy, 20 (53 per cent) achieved a complete remission. The actuarial median survival of all patients was 17 months. Age greater than 60 years and stage III/IV disease predicted a poor clinical outcome, whereas the large cell histological subtype predicted a favourable outcome. Prospective clinical studies using uniform treatments and a uniform histologic classification scheme are needed to confirm these findings.     

Renal involvement in patients with non-Hodgkin's lymphoma: clinical and pathological features in 23 cases

Twenty-three cases of renal or perirenal involvement by non-Hodgkin's lymphoma were identified from a retrospective review of computed tomography (CT) scans performed on patients at St Bartholomew's Hospital over an 8-year period. The histology, clinical features and survival of these patients were examined. Eighteen of the 23 patients had high grade histological subtypes according to the Kiel classification. The majority of cases were identified from scans performed at the time of presentation. In nine cases renal lymphoma was observed in the absence of detectable retroperitoneal lymphadenopathy. Staging was rarely altered by the finding of renal lymphoma as 21 of the 23 patients had involvement of other extranodal sites in addition to the kidney. Survival did not appear to be adversely affected by the presence of renal lymphoma at the time of initial diagnosis. During the same period no cases of renal involvement were identified in patients with Hodgkin's disease, although one patient had perirenal involvement.     

71例原发性中枢神经系统淋巴瘤的临床、影像学与病理学特征

背景与目的：原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma, PCNSL）是临床少见的非霍奇金淋巴瘤,近年来发病率有所上升.本研究的目的是探讨PCNSL的临床、影像学与病理学特征,提高临床对PCNSL的认识.方法：回顾性分析经病理证实的71例PCNSL的临床资料.结果：71例患者中男性45例,女性26例,男女比例为1.73：1;其中50岁以上患者44例（62.0%）,平均年龄52岁;就诊时间在1个月内者51例（71.8%）;临床症状多表现为颅内压力增高及局灶性神经系统损害的定位体征;单发病灶47例（66.2%）;MRI示T1WI低信号或等信号,T2WI高信号或等信号,大多数病灶显像增强后呈明显强化;所有病例病理均为B细胞性.结论：PCNSL多发于中老年人,男性多见,病程短,单发病灶多见,病灶多呈明显均匀强化,绝大多数为B细胞性,临床表现及影像学缺乏特异性,确诊需依赖于病理.     

Meningeal dissemination in primary CNS lymphoma: diagnosis,treatment, and survival in a large monocenter cohort

The frequency of meningeal dissemination (MD) in primary CNS lymphoma (PCNSL), its prognostic impact, and optimal management have not been defined thus far. In 69 of 92 (75%) immunocompetent patients, primarily diagnosed with PCNSL at our institution between January 1994 and February 2007, cerebrospinal fluid was analyzed for MD. MD was found by cytomorphology in 7/63 (11%), by immunophenotyping in 1/32 (3%), and by PCR of the IgH CDR III region in 6/37 (16%). Neuroradiologic examination revealed MD in 3 of 69 patients (4%). Median event-free survival (EFS) of patients with MD diagnosed by any of the methods was 26 months, of those without MD 34.1 months (P = .24); median overall survival (OAS) of these two patients'' groups was 45.5 and 42.5 months, respectively (P = .34). Patients with cytomorphologic proof of MD had a median EFS of 15.4 months and OAS of 18.5 months, those without MD 34.3 and 45 months (P = .018 and .017, respectively). We found a low frequency of MD despite the use of putatively sensitive diagnostic methods. No impact on outcome was seen for MD, diagnosed by any of the methods used; however, patients with cytomorphologic proof of MD had a significantly shorter median EFS and OAS.     

42例恶性淋巴瘤的超声诊断及临床分析

目的探讨恶性淋巴瘤的声像图表现及超声检查的临床意义.方法对42例恶性淋巴瘤进行彩超、临床、病理分析.结果恶性淋巴瘤可发生于全身一个或多个部位,表现为单纯淋巴结肿大和多个脏器受累,前者声像图为界限清晰的堆积或排列状低回声结节,后者为该脏器肿大,内见无包膜的片状或境界清晰的结节状回声.结论超声检查对恶性淋巴瘤的临床诊断、治疗具有重要意义.     

42例原发性中枢神经系统淋巴瘤患者预后影响因素分析

背景与目的：原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma,PCNSL）是发生在脑、脊髓、脑膜或眼的罕见侵袭型非霍奇金淋巴瘤,无CNS之外的部位累及。PCNSL与其他类型淋巴瘤相比,患者生存期短,预后差,且复发率高,未经治疗的患者的中位生存期仅为3个月。近年来研究发现C-MYC、BCL-2、BCL-6、Ki-67等指标在一定程度上影响PCNSL患者预后。因此,通过分析PCNSL相关蛋白表达、治疗方式及其他临床因素对患者预后的影响, 希望为该病的临床治疗及预后评价进一步积累资料。方法：回顾性分析自2013年6月—2021年5月于大连医科大学附属第二医院治疗的42例经病理学检查明确诊断为原发性中枢神经系统弥漫大B细胞淋巴瘤患者的临床资料,包括性别、年龄、病灶数量、美国东部肿瘤协作组（Eastern Cooperative Oncology Group,ECOG）评分、血清乳酸脱氢酶（lactate dehydrogenase,LDH）、病灶是否累及深部脑组织、治疗方案、病理学Hans分型及C-MYC、BCL-2、BCL-6、Ki-67等生物标志物,结合随访调查,了解患者生存时间及生存状况,应用Kaplan-Meier法及log-rank检验分析影响患者无进展生存期（progression-free survival,PFS）和总生存期（overall survival,OS）的预后相关因素,多因素分析采用COX回归模型。结果：42例PCNSL患者中位发病年龄61岁,男女比例为1.33∶1.00,颅脑增强MRI病灶多呈均匀明显强化。所有患者均接受含有大剂量甲氨蝶呤（high-dose methotrexate,HD-MTX）方案化疗,治疗后评价完全缓解（complete response,CR）20例、部分缓解（partial response,PR）5例,疾病稳定（stable disease,SD）11例,疾病进展（progressive disease,PD）6例。中位PFS为21个月,中位OS为34个月,1年PFS率为63.7%,2年PFS率为47.0%;1年OS率为70.8%,2年OS率为55.6%。单因素分析结果显示,影响PFS的因素是HD-MTX多药联合化疗、鞘内化疗及联合利妥昔单抗。影响OS的因素是ECOG评分≥2、C-MYC（+）、BCL-2及C-MYC双表达、HD-MTX多药联合化疗、鞘内化疗及联合利妥昔单抗。多因素分析结果显示：利妥昔单抗治疗是影响PFS的独立预后因素（P=0.020）,ECOG评分、利妥昔单抗是影响OS的独立预后因素（P=0.007;P=0.046）。与未接受巩固治疗的患者相比,接受巩固治疗患者的中位PFS及OS较高;进一步的亚组分析显示,自体干细胞移植（autologous stem cell transplantation,ASCT）组的中位PFS及OS较全脑放疗（whole brain radiation therapy,WBRT）组高,但差异无统计学意义。结论：PCNSL多发于中老年人,男性多于女性,影像学缺乏特异性。ECOG评分≥2与PCNSL患者较差的OS相关。C-MYC（+）、BCL-2及C-MYC双表达可作为指导危险分层的预后标志物。以HD-MTX为基础的多药联合化疗已经成为PCNSL的首选治疗手段,利妥昔单抗的应用可延长生存期。在全身化疗的基础上,联合局部鞘内化疗可以改善预后。进一步的巩固治疗主要包括ASCT及WBRT,可延长PFS及OS,ASCT可以取得与WBRT相似的疗效,且可避免WBRT的晚期神经毒性,但本研究中因样本量及随访时间的限制,未得出明确的统计学结果。     

Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus-positive diffuse large B-cell lymphoma,NOS of bone marrow-liver-spleen type: an autopsy case report

Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) has a significantly poor prognosis among secondary HLH. We describe the rare case of a 74-year-old female with secondary HLH presenting with a rapidly fatal course. Post-mortem examination revealed Epstein-Barr virus (EBV) -positive diffuse large B-cell lymphoma (DLBCL). We were unable to make a definite antemortem diagnosis because the patient did not exhibit lymphadenopathy and bone marrow biopsy demonstrated hemophagocytosis without evidence of lymphoma. She died of multiple organ failure on the twelfth day of hospitalization despite a temporary response to steroids. Autopsy revealed diffuse lymphoma cell infiltration of the bone marrow, liver and spleen, suggesting “bone marrow-liver-spleen” (BLS)-type large B-cell lymphoma (LBCL). BLS-type LBCL is a rare and clinically aggressive lymphoma, usually associated with fever, cytopenia and HLH. The disease has a high mortality rate due to the delay in diagnosis and a highly aggressive clinical course. Further studies are required to improve our understanding of this rare extranodal DLBCL.     

胃癌腹膜转移的临床病理特性及预后分析

目的 探讨胃癌腹膜转移(P2)的临床病理特性及不同术式对预后的影响。方法 对91例P2患者的临床病理资料及不同术式术后生存情况进行回顾性分析。结果 单因素分析发现,肿瘤大小、肿瘤部位、分化程度、Borrmann分型、脏器侵犯、淋巴结转移、浸润深度、肝转移与P2相关(P＜0.05)。Logistic回归分析显示,浸润深度和脏器侵犯是P2独立的影响因素(P＜0.05)。非根治切除术组和姑息手术组在年龄、腹水及脏器侵犯上差别有统计学意义(P＜0.05)。姑息手术组中造瘘术、胃肠吻合术与剖腹探查术间的生存时间差别无统计学意义(P＞0.05)。非根治切除术组的中位生存时间为13.4月,姑息手术组的中位生存时间为4.2月,差别有统计学意义(P＜0.01),Cox分析显示手术方式和肿瘤大小是影响P2患者预后的独立危险因素。结论 胃癌患者的临床病理特征与腹膜转移的发生密切相关,非根治切除术可改善胃癌腹膜转移(P2)患者的预后。     

肺癌胸膜种植转移的CT表现及其解剖分布

目的 总结胸膜种植转移的CT征象及其解剖分布。方法 回顾分析32例临床、病理确诊为原发性肺癌伴胸膜种植转移患者的CT表现。结果 本组患者的胸部CT征象主要表现为胸腔积液(24例)、脏层胸膜转移结节(10例)、壁层胸膜转移结节(16例)及胸膜增厚(3l例)。脏层胸膜转移结节中，分布于肺表面脏层胸膜9处，叶间胸膜l0处。壁层胸膜转移结节分布在膈胸膜、肋胸膜、纵隔胸膜、肺韧带，共45处。结节小至2—5mm的粟粒，大至5～10mm。胸膜增厚中因直接侵犯造成者10例，间接转移者2l例，后者中9例表现为增厚≤10mm，4例一侧胸膜环状增厚，5例纵隔胸膜增厚，3例肺韧带增厚。结论 肺癌胸膜转移最常见的CT征象为胸腔积液，其次为胸膜转移结节及胸膜增厚。转移结节最常分布在隔胸膜、肋胸膜，并可转移至肺韧带；早期表现为粟粒状，在肺窗容易发现。     

原发性结外淋巴瘤42例临床及误诊分析

目的：探讨结外型恶性淋巴瘤临床特点、误诊原因及早期诊断和治疗。方法：对经病理确诊的结外非霍奇金淋巴瘤42例进行回顾性分析。结果：误诊率66．7％，发病部位以韦氏环、鼻腔最多见，其次为皮肤和胃肠道：29例行综合治疗，手术 化疗和／或放疗22例(5例联合生物治疗)，治疗 化疗7例；随访40例(95．2％)，尚有24例健在，生存5年以上者8例，Ⅰ、Ⅱ期占75．0％。结论：结外型淋巴瘤易误诊，临床表现缺乏特异性，韦氏环、鼻腔及皮肤最易受侵犯，病理检查是确诊的唯一方法；早期诊断、早期治疗预后较佳。     

Treatment of CNS dissemination in systemic lymphoma

Summary The frequency of central nervous system (CNS) dissemination in non-Hodgkin’s lymphoma (NHL) varies and is dependent on NHL histology. More than 50% of patients with CNS involvement have advanced and progressive systemic disease. While CNS involvement at initial diagnosis may be treated curatively, treatment of CNS involvement in systemic relapsing or refractory lymphoma is challenging and most often palliative. Due to a paucity of randomized trials, treatment of lymphomatous metastases is not standardized. Nonetheless, treatment of LM entails administration of both CNS-directed and systemic chemotherapy that often includes high-dose chemotherapy regimens with stem cell support. Gleissner and Chamberlain contributed equally.     

Prognostic value of MYC rearrangement in cases of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma

BACKGROUND:

B‐cell lymphoma, Unclassifiable with features intermediate between diffuse large B‐cell lymphoma (DLBCL) and Burkitt lymphoma, for convenience referred to here as unclassifiable B‐cell lymphoma, is a category in the 2008 World Health Organization system used for a group of histologically aggressive neoplasms that are difficult to classify definitively. Currently, there is no established standard therapy for these neoplasms.

METHODS:

The authors assessed MYC status and correlated it with treatment response and outcome in a group of 52 patients with unclassifiable B‐cell lymphoma treated with either a standard DLBCL regimen (R‐CHOP [rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone‐related therapy]) or more intensive regimens, such as R‐hyper‐CVAD (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high‐dose methotrexate and cytarabine). The regimens were selected by the treating clinicians based on the overall clinical and pathological findings.

RESULTS:

Thirty (58%) unclassifiable B‐cell lymphomas had MYC abnormalities (MYC⁺) including 27 with rearrangement, 2 with amplification, and 1 with both. The MYC⁺ and MYC^? groups were similar in their age distribution and International Prognostic Index scores. Progression‐free survival of patients with MYC⁺ unclassifiable B‐cell lymphoma treated initially with R‐CHOP was significantly worse than patients treated with R‐hyper‐CVAD (P = .0358). In contrast, for the MYC^? unclassifiable B‐cell lymphoma group, some patients responded to R‐CHOP, and others were refractory to R‐hyper‐CVAD.

CONCLUSIONS:

MYC aberrations are common in unclassifiable B‐cell lymphoma. The presence of MYC aberrations identifies a patient subset that requires more aggressive therapy than R‐CHOP. In contrast, MYC^? unclassifiable B‐cell lymphoma patients responded variably to either R‐CHOP or aggressive therapy, and the latter showed no survival advantage. Cancer 2011;. © 2011 American Cancer Society.     

胃原发性恶性淋巴瘤内窥镜活检病例的临床病理特点

目的:探讨胃原发性恶性淋巴瘤黏膜活检病例的临床病理诊断特点.方法:内窥镜胃黏膜活检标本和切除标本经常规石蜡包埋切片、染色和免疫组化染色后光镜下观察.结果:31例活检组织中均呈现不同程度的异型淋巴细胞弥漫浸润,腺体数量减少或完全消失;12例出现淋巴上皮病变,7例出现可疑淋巴上皮病变;13例伴有炎症渗出坏死;8例组织挤压损伤.结论:充分、准确的活检对胃淋巴瘤的确诊具有重要意义;异型淋巴细胞弥漫浸润是活检诊断的主要特征,活检组织中可能不出现淋巴上皮病变;免疫组化染色和分子生物学技术可以提高诊断的准确率;内镜医师和病理医师间的合作是提高诊断率的关键.     

Non-Hodgkin's lymphoma of the pleural cavity developing from long-standing pyothorax. Summary of clinical and pathological findings in thirty-seven cases

Our previous study suggested a close relation between a preceding chronic tuberculous pyothorax and the development of non-Hodgkin's lymphoma (NHL) in the pleural cavity. To confirm this further, 37 cases were collected from Japanese hospitals, and their clinical and pathological findings summarized. The age at first admission for lymphoma of patients ranged from 46 to 81 (mean 63) years, the male to female ratio being 5.2:1. All patients were admitted after a 22-55 (mean 33) year history of pyothorax resulting from artificial pneumothorax for the treatment of pulmonary tuberculosis (29 cases) or tuberculous pleuritis (seven cases). The most common presenting symptom was chest pain. The main tumor mass, detected by chest roentgenogram and computed tomographic scans, was situated in the pleura (28 patients), the lung near the pleura (five patients) and the pleura and lung (four patients). The diagnosis of pleural NHL was made by biopsy for 31 of the patients and at autopsy for the other six. Histologically 30 (81%) of the 37 cases were of the diffuse large cell type, and of these the immunoblastic type was the most common (22 cases). Immunological and immunohistologic studies revealed a B-cell nature of the proliferating cells in all but one tumor. Thirty-two patients received chemotherapy and/or radiotherapy. Twenty-seven patients died between one and 144 (median eight) months of diagnosis. Autopsies carried out in 23 cases revealed the disease to have been localized to the thorax in 11 patients. These findings indicated that malignant B-cell lymphoma arose as a monoclonal growth from a pool of proliferating polyclonal B lymphocytes in tissues affected by the chronic tuberculous pyothorax.     

Clinical and Pathological Features of Primary Gastrointestinal Non-Hodgkin＇s Lymphoma

OBJECTIVE The study was initiated to obtain histologic distribution, clinical features, and treatment results in patients with primary gastrointestinal non-Hodgkin‘s lymphomas.METHODS Between January 1990 and January 2000, 89 PGI NHL patients were eligible to evaluate clinical features. Histological and immunohistological studies were routinely used and all the specimens were reclassified according to the recently published WHO classification system.RESULTS (1)Clinically, among the 89 patients, there were 24 patients in stage IE,33 in stage IIE,19 in stage IIIE,and 13 in stage IVE. (2)Immunohistological studies revealed 72 patients were with B-cell type and only 17 with T-cell type. (3)Altogether, 15 MALT lymphoma were diagnosed among 89 PGI NHL patients, and 14/15 were found primary in the stomach.(4)The 3-year and 5-year overall survival were 77.0% (57/74) and 53.6% (30/56)for the total group.CONCLUSION No clinical symptoms and signs were found to be specific for the diagnosis of PGI NHL. Most patients were in stage IE and liE when diagnosed and the intermediate grade and B-cell type were more common than the others. Surgical resection of the tumor and standard combined chemotherapy post surgery were suggested to be the most effective measures for the long term survival of the PGI NHL patients.