Primary non-Hodgkin's lymphoma of the intestine: a morphological, immunohistochemical and clinical study of 31 Chinese cases

Thirty-one cases of primary non-Hodgkin's lymphoma of the intestine were investigated. Twenty-one were of B-cell and 10 of T-cell origin. The B-cell lymphomas comprised two cases of low-grade B-cell lymphoma of mucosaassociated lymphoid tissue (MALT), one of centroblastic/centrocytic type, three of high-grade B-cell lymphoma coexisting with a low-grade B-cell lymphoma of MALT, nine of centroblastic, three of immunoblastic and three of Burkitt type. Of the T-cell lymphomas, eight were of pleomorphic medium-to large-sized cell type and two of large cell anaplastic type. All the B-cell lymphomas expressed CD20 (L26) and/or Ki-B5; in six there was monotypic immunoglobulin light chain restriction. Membrane positivity for CD45RO (UCHL1) was observed in the 10 cases of T-cell lymphoma, but the tumour cells did not express monocyte-macrophage markers. Clinically, the patients with T-cell lymphomas were usually young males with constitutional symptoms and their prognosis was significantly worse than those of patients with intestinal B-cell lymphoma.     

8例大肠原发性恶性淋巴瘤诊治临床分析

目的 为了探讨原发性大肠恶性淋巴瘤的诊断和治疗。方法 回顾了我院1983年－1999年收治的8例原发性大肠恶性淋巴瘤的临床资料，并结合有关文献对原发性大肠恶性淋巴瘤的临床特点。诊断及预后进行了分析。结果 8例患者均行手术探查，其手术切除率为100％，其中根治手术达75％。5年生存率为37．5％。结论 提高大肠原发性恶性淋巴瘤的治愈率和生存期的关键是对本病的临床特点和病理特点的认识，作到早发现，早诊断。治疗上采取以手术为主的综合疗法。     

Primary malignant lymphomas of the central nervous system: a histological and immunohistological study of 12 cases

Paraffin sections of surgical and autopsy material from 12 cases of primary non-Hodgkin's lymphomas of the central nervous system were examined for histopathological diagnosis and for the demonstration of cytoplasmic immunoglobulins. According to the Kiel classification, there were five cases of lymphoplasmacytoid polymorphous lymphoma, five of immunoblastic lymphoma, one of lymphoblastic lymphoma of convoluted cell type. There was also one of the recently described multilobated lymphoma. An immunohistological study of light and heavy chains by peroxidase-antiperoxidase (PAP) technique and avidin-biotin complex (ABC)technique was performed. Intracellular immunoglobulins were demonstrated in seven cases: four cases were classified as immunoblastic lymphomas and three cases as lymphoplasmacytoid lymphomas. Negative immunoglobulin staining was observed in five cases: two lymphoplasmacytoid lymphomas, one immunoblastic, one lymphoblastic of convoluted cell type and one multilobated. A 'monoclonal' pattern of immunoglobulin staining was detected in six cases. One case, classified as immunoblastic lymphoma, showed 'bitypic' staining for kappa and lambda chains. It was concluded that primary CNS non-Hodgkin's lymphomas of the present series showed morphological and immunohistological features similar to those of malignant lymphomas arising in extraneural sites. In particular, the presence in our series of a multilobated lymphoma, as a primary CNS tumour, is emphasized.     

Peripheral T-cell lymphoma: a clinicopathological study of 41 cases and evaluation of the prognostic significance of the updated Kiel classification

A total of 41 non-cutaneous peripheral T-cell lymphomas were classified following the updated Kiel classification. Of these, 20 cases belonged to the low-grade group (T-cell chronic lymphocytic leukaemia, 3; lymphoepithelioid, 5; angioimmunoblastic, 4; pleomorphic small cell, 8) and 21 to the high grade group (pleomorphic medium and large cell, 11; immunoblastic, 3; large-cell anaplastic Ki-1 positive, 7). Seventy per cent showed a CD4+/CD8-phenotype, 39% a defective phenotype and 88% an activation phenotype. Eighty per cent had B-symptoms, 63% hepatomegaly, 48% splenomegaly and 26% had involvement of more than three lymphoid areas. Bone marrow was infiltrated in 34% central nervous system in 4%, lung in 12% and skin in 14.6%. Seventeen per cent presented with extranodal disease and 82.8% had stage III/IV disease. Hypergammaglobulinaemia was found in 29%, hypercalcaemia in 7%, raised LDH serum levels in 58% and HTLV-I antibodies in only one case. Of the 37 treated patients 18 (48%) achieved a complete remission, but 33% relapsed. Mortality was 59% and actuarial overall survival at 38 months was 0.32. In the comparison of the clinical, analytical and immunophenotypic variables and outcome between low and high grade groups, only the average of bone marrow infiltration in the low grade and stage I–II, presence of defective phenotypes and higher Ki-67 positivity in the high grade group were significantly different. In the statistical studies, extranodal prentation and the failure to achieve a complete remission were the only variables that influenced mortality; there weere no significant differences in the general features of the low and high grade groups and only minor differences were found in the immunoblastic and angioimmunoblastic subgroups. There were no differences in the actuarial survival between the low and high grade groups, among the subgroups of the Kiel classification, among stages I to IV, between patients with or without B-symptoms, with or without defective phenotypes, Ki-67 positivity over or under 60%, or among different CD4/CD8 phenotypes. The updated Kiel classification did not separate groups with a prognostic significance.     

原发性皮肤恶性淋巴瘤的临床病理和免疫组化研究

研究原发性皮肤恶性淋巴瘤的类型及临床病理特点,方法对４２例ＰＣＭＬ作了形态学观察,免疫表型标记和组织学分型。结果原发性皮肤Ｔ细胞性淋巴瘤（ＰＣＴＣＬ）占６９．０％,其中蕈样霉菌病占７．２％（ＭＦ）、血管中心性淋巴瘤占９．５％皮下指明脂膜炎样Ｔ细胞巴瘤占１９．０％,原发性皮肤Ｂ细胞性淋巴瘤（ＰＣＢＣＬ）占３１．０％;ＰＣＭＬ男女之比为２：１,中位年龄３７岁,ＰＣＴＣＬ的发病年龄小于ＰＣＢＣＬ（     

Primary malignant lymphoma of the large intestine complicating chronic inflammatory bowel disease

Ten cases of malignant lymphoma of the colon and rectum complicating chronic inflammatory bowel disease are presented. Seven patients had chronic ulcerative colitis with a history varying from 6 to 20 years. There was extensive colitis in six of these patients and left-sided colitis in one. All seven lymphomas showed the pathological and immunohistological features of primary B-cell tumours of the gastrointestinal tract with a predominance of high-grade tumours. Three patients had Crohn's disease of the large intestine complicated by malignant lymphoma of the sigmoid colon or rectum. The history of Crohn's disease varied from 30 months to 20 years and in each case there was fissuring and fistulae. There was extensive anal involvement in two cases. Histologically the three lymphomas were heterogeneous: one was of 'granulomatous' T-cell type and the other two were markedly polymorphic and of equivocal phenotype. They were also characterized by numerous multinucleate tumour giant cells. Primary colorectal malignant lymphoma should be regarded as a rare, but significant, complication of ulcerative colitis. Immunosuppression may be an additional factor in the genesis of intestinal lymphoma in Crohn's disease. The prognosis appears to be dependent on factors already known to be of prognostic significance in primary gut lymphomas: a predominance of high-grade tumours suggests that the outlook is generally worse than that for idiopathic primary large intestinal lymphoma.     

Primary malignant lymphoma of the thyroid-a tumour of mucosa-associated lymphoid tissue: review of seventy-six cases

Seventy-six cases of primary thyroid lymphoma have been reviewed employing the Kiel classification. These lymphomas are almost entirely of follicle centre cell origin, occur predominantly in elderly females, and are frequently associated with lymphocytic thyroiditis or Hashimoto's disease. The overall prognosis is variable, with long-term survival in a substantial number of cases following thyroidectomy and radiotherapy. Results of histological examination including immunoperoxidase studies are described. It is proposed that thyroid lymphoma represents a neoplasm of mucosa-associated lymphoid tissue (MALT), and the implications of this are discussed.     

Cytodiagnosis of non-Hodgkin's lymphoma

Summary A cytomorphological analysis using a large number of variables was applied to 136 fine needle aspirations and 122 imprints from 258 biopsy proven lymphoid lesions, including 203 non-Hodgkin's lymphomas (NHL), 12 true histiocytic neoplasms and 43 lymphoid hyperplasias. Characteristics of the various cell types are described using the Kiel classification predominantly.Two blindly performed consecutive cytological analyses, indicated as Cyt I and Cyt II respectively, were compared with histology. False-positive conclusions did not occur. False-negative conclusions were present in 3.7% of cases. The intra-observer reproducibility between Cyt I and Cyt II was 93%.The inter-observer reproducibility between the 4 authors was examined in 50 cases; consensus regarding malignancy, exact NHL type and benign cytology was 88%, 70% and 100% respectively. Cytodiagnosis of NHL is a reliable method with a high sensitivity which can add substantial information to tissue diagnosis in troublesome cases.Fine needle aspirations, if adequately performed, had the same cellularity, quality and cellular composition as imprints. Aspiration cytology is very useful for the selection of a representative lymph node for surgical biopsy, for the diagnosis of recurrent NHL, for staging the extent of disease, for the diagnosis of cases in which tissue biopsy is not possible, and as a variable for monitoring treatment.Abbreviations Cyt I first blind cytological screening - Cyt II second blind cytological screening - FNA fine needle aspiration - l.g.b. lymphoglandular body - LH lymphoid hyperplasia - MHT malignant histiocytic tumour - NHL non-Hodgkin's lymphoma An editorial will follow giving comment on the fundamental question of the value of aspiration cytology in lymph node pathology.     

Primary small intestinal lymphoma: a study of 39 cases

Thirty-nine cases of primary small intestinal lymphoma were studied by morphological and immunohistochemical methods. The adjacent uninvolved mucosa was also examined for features suggestive of coeliac disease. Employing the immunogold silver staining (IGSS) technique and polyclonal primary antisera against alpha-l-antitrypsin, lysozyme and kappa and lambda light chains, 29 cases (74.5 per cent) were found to be B-cell lymphomas, seven (18 per cent) histiocytic tumours, one (2.5 per cent) Hodgkin's disease and two (5 per cent) remained unclassified. No specific lymphoma subtype was found to be associated with cases having the histological features of coeliac disease in the uninvolved adjacent mucosa. In 35 cases sufficient clinical information was available to assess the significance of histological type and stage in relation to survival. Although the histological type did not correlate with survival, stage did and tumours extending beyond the local lymph nodes were associated with a significantly worse prognosis.     

443例恶性淋巴瘤临床病理特征分析

目的：根据WHO(2008版)造血和淋巴肿瘤分类标准,探讨军事医学科学院附属医院恶性淋巴瘤的病理类型及分布特点。方法：收集2010年11月1日至2015年3月31日病理诊断443例恶性淋巴瘤,复习其临床资料、HE切片及免疫组织化学切片。按WHO(2008版)分类标准进行病理诊断及分类。结果：443例恶性淋巴瘤中霍奇金淋巴瘤11.06%(49/443),非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL)88.94%(394/443)。非霍奇金淋巴瘤中B细胞性淋巴瘤66.37%(294/443),T细胞性淋巴瘤22.35%(99/443)。非霍奇金淋巴瘤中,发病构成比居前5位分别为弥漫性大B细胞淋巴瘤41.53%(184/443)、滤泡性淋巴瘤11.06%(49/443),外周T细胞淋巴瘤5.64%(25/443),T淋巴母细胞白血病/淋巴瘤5.19%(23/443)、黏膜相关淋巴组织结外边缘区淋巴瘤4.74%(21/443)。HL中以结节硬化型经典型霍奇金淋巴瘤为多(4.51%,20/443)。恶性淋巴瘤患者中,男女比例为1.58∶1,中位年龄57岁(7~94岁)。霍奇金淋巴瘤和非霍奇金淋巴瘤起病部位均以颈部淋巴结为多。结论：443例淋巴瘤中,非霍奇金淋巴瘤发病远多于霍奇金淋巴瘤,非霍奇金淋巴瘤中以弥漫大B细胞淋巴瘤多见,霍奇金淋巴瘤中以结节硬化性霍奇金淋巴瘤多见。大部分淋巴瘤类型以男性发病为多,起病部位以颈部淋巴结为多。     

Primary spinal epidural manifestation of malignant lymphoma

The clinical, histological and immunomorphological data in 19 cases of primary spinal epidural manifestation of malignant lymphomas collected between 1974 and 1994 are reported. The age of the patients varied between 11 and 87 years with a mean age of 56.3 years. There was a slight male predominance (11 : 8). In most cases, the onset of the clinical symptoms was rapid. The preferential tumour localization was the epidural space related to the thoracal vertebral bone. In each case, decompressive laminectomy was performed. The tumours were histologically and immunomorphologically classified as B-cell lymphomas (14 of 19), T-cell lymphomas (3 of 19) and anaplastic plasmacytoma (1 of 19). Except for one case, post-operative staging did not reveal anything other than epidural manifestation of the malignant lymphoma. The vertebral bone, however, was involved in seven cases. Irradiation alone, or in combination with chemotherapy, was performed as additional therapy. The post-operative survival time was variable.     

Chromosomal translocation detected by bcl-1 and bcl-2 rearrangement in low-grade B-cell lymphomas in a European population

Twenty-nine cases of non-Hodgkin's lymphoma of low-grade malignancy in a European population were investigated for the presence of bcl-2 and bcl-1 gene rearrangement. The cases were classified according to the Kiel classification. It was shown that bcl-2 gene rearrangements were exclusively confined to centroblastic-centrocytic lymphomas. bcl-1 rearrangements were found in two cases of chronic lymphocytic leukaemia. As the chromosomal translocation t(14;18) is reported to occur in up to 85% of follicular lymphomas, our results provide additional evidence that the differentiation of low-grade B-cell lymphomas according to the Kiel classification defines biologically distinct entities.     

Lung carcinoma mimicking malignant lymphoma: Report of three cases

Three cases of lung carcinomas with unusual histologic appearances that have received little or no comment in the literature are presented. They were initially confused with malignant lymphoma because of a diffuse proliferation of relatively monotonous cells simulating large-cell immunoblastic lymphoma. In each case, the possibility of malignant lymphoma was excluded with confidence after the immuno-histochemical study (leucocyte common antigen negative and cytokeratins positive), although with conventional microscopy several foci of cohesive groups of tumor cells were observed. The tumors were ranked at the clinical stage II or III when they were initially discovered, but all patients died of disease within 1 year. The present three tumors show an aggressive behavior and could be classified into a peculiar variant of ‘large cell’ carcinoma. It is necessary for surgical pathologists to have an idea of these variants of lung carcinoma in order to avoid erroneous diagnosis.     

脾脏非霍奇金淋巴瘤临床病理学研究

目的 探讨脾脏非霍奇金淋巴瘤(SNHL)的形态学特点、免疫表型及鉴别诊断。方法 对39例SNHL进行形态学观察及免疫组化ABC法分析，使用抗体包括CD3、CD45RO、CD56、CD79α、CD20、CD68、Mac387、CD5、CD10、bcl—2、CD23、CD43、cyclinD1、IgM、IgD等。结果 39例SNHL中有B细胞淋巴瘤(BCL)24例，包括小淋巴细胞性淋巴瘤(SLL)4例，套细胞淋巴瘤(MCL)4例，滤泡性淋巴瘤(FL)5例，边缘区淋巴瘤(MZL)6例，弥漫大B细胞性淋巴瘤(DLBCL)5例，其中2例为大多叶核细胞淋巴瘤。T细胞淋巴瘤(TCL)11例，其中肝脾T细胞淋巴瘤2例，非特异性TCL9例。组织细胞性淋巴瘤4例。结论 脾脏淋巴瘤病理类型多样，掌握其形态学特征，熟悉各类型的免疫表型的异同点对诊断与鉴别诊断有重要意义。     

Malignant lymphomas and undifferentiated small cell carcinoma of the thyroid: a clinicopathological review in the light of the Kiel classification for malignant lymphomas

The paper reports a re-evaluation--based on the Kiel classification for non-Hodgkin lymphomas--of a group of cases initially diagnosed as undifferentiated small cell carcinomas or primary lymphomas of the thyroid. Twelve such cases were found among the 155 cases of primary malignant tumours of the thyroid recorded at the Institut Jules Bordet between 1955 and 1975. The review of the clinical charts and the histology showed that all the cases were in fact malignant lymphomas fitting easily into one of the groups described in the Kiel classification. These findings support the growing opinion that undifferentiated small cell carcinoma of the thyroid does not exist as a distinctive clinicopathological entity. Furthermore, the Kiel classification proved to be an excellent prognostic indicator, since all the cases classified as highly malignant were indeed fatal, whereas the surviving cases--three of which had shown tumoral extension beyond the thyroid capsule--fell into the group of low malignancy. Lastly, this study acknowledges the frequently observed association of malignant lymphoma of the thyroid with stigmata of Hashimoto's disease, and thus supports the concept that the continuous antigenic stimulation observed in the latter could trigger the development of a malignant lymphoma.     

Distribution of malignant lymphoma in Japan: analysis of 2260 cases, 2001-2006

The World Health Organization classification was used to conduct an analysis of geographic, age, sex, and lesion primarily biopsied/resected distribution of 2260 lymphoid neoplasms diagnosed during 2001–2006 throughout Japan. B-cell neoplasms accounted for 65% of all lymphoid neoplasms, T/natural killer (T/NK)-cell neoplasms for 25% and Hodgkin lymphoma for 7%. The most common type was diffuse large B-cell lymphoma (DLBCL, 33%), followed by follicular lymphoma (18%), and adult T-cell leukemia/lymphoma (ATLL, 10%). The high rate of 18% for follicular lymphoma was similar to that in Western countries (11–33%). T/NK-cell neoplasms accounted for a higher percentage of lymphoid neoplasms in Kyushu (30%) and Okinawa (38%) compared with other areas of Japan (18–20%). Among T/NK-cell neoplasms, ATLL was the most common type in Okinawa (54%) and Kyushu (59%). Extranodal NK/T cell lymphoma was the second most common type of T/NK-cell neoplasms in Okinawa (15%). This epidemiological study shows that the distribution patterns of malignant lymphoma differ especially in Kyushu and Okinawa, the endemic area of human T-cell leukemia/lymphoma virus type 1.     

贵州地区淋巴瘤类型分布

使用免疫组化法分析２５３例恶性淋巴瘤，应用改良的Ｔ、Ｂ非何杰金淋巴瘤（ＮＨＬ）的分类法进行分类。Ｂ细胞性淋巴瘤１３７例，占５４．１５％。其中慢性淋巴细胞性（１４．４％），小裂细胞性（１８．２５％），裂—无裂细胞性（２０．４４％），大裂细胞性（９．４９％），小无裂细胞性（５．１１％），大无裂细胞性（８．７６％）．Ｔ细胞淋巴瘤１１６例（４５．８５％）：其中慢性淋巴细胞性（１０．３４％），多形性（小细胞性）（２７．５９％），多形性（中细胞性）（１６．３８％），多形性（大细胞性）（６．０３％），透明细胞性（７．７６％），淋巴母细胞性（８．６２％）。两组各占７６％。结果说明改良法是适合中国淋巴瘤分类的。     

Primary lymphoma of the thyroid: a clinical, histological and immunohistochemical study of 20 cases

Twenty cases of malignant lymphoma arising in the thyroid gland were studied clinically, histologically and immunohistochemically. Nineteen cases were non-Hodgkin's lymphoma (15 diffuse and four follicular lymphoma) and one was a plasmacytoma. Immunohistochemical analysis of the lymphomas using paraffin-embedded sections disclosed that 17 lymphomas were B-cell type and two were T-cell type. The plasmacytoma was of IgG kappa type. The large majority of the lymphomas were associated with an underlying chronic thyroiditis. The 5-year survival rate of the patients was 70%. An unfavourable diagnosis was more likely when the tumour was diffuse rather than follicular, when it was of diffuse large cell type or of immunoblastic type and when there was cervical lymph node involvement.     

Primary malignant lymphoma of the colon and rectum. A histopathological and immunohistochemical analysis of 45 cases with clinicopathological correlations

Primary colorectal lymphoma accounts for only about 0.2% of large intestinal malignancies. Management difficulties are compounded by a lack of consistent pathological reporting and by the use of numerous different classifications. Forty-five cases of primary colorectal lymphoma are included in this study. The presenting features are indistinguishable from those of colorectal cancer. Seven patients had a history of chronic ulcerative colitis but no other predisposing factors were identified. Immunohistochemical studies showed that all tumours were of B-cell phenotype. Most tumours were difficult to characterize using standard pathological classifications such as Kiel, except for the 11 cases of malignant (multiple) lymphomatous polyposis, which were morphologically diffuse centrocytic lymphomas. Twenty-nine lymphomas showed the morphological and immunohistochemical features associated with tumours arising in mucosa-associated lymphoid tissue. These tumours showed variable but often marked polymorphism and we have used the term polymorphic B-cell lymphoma to describe them. Consistency was achieved between three observers as to whether these tumours were low or high grade, and grade was found to be prognostically important. A modified Dukes staging system was adopted and there was a trend for early stage to give prognostic advantage. This study supports the view that surgery should be the primary treatment for localized lymphoma of the gastrointestinal tract with radiotherapy and/or chemotherapy for advanced cases and for malignant lymphomatous polyposis.