Primary Extranodal Non-Hodgkin’s Lymphoma: Clinicopathological Features,Survival and Treatment Outcome in Two Cancer Centers of Southern Turkey

Background: The aim of this study was to assess the epidemiological and clinicopathological characteristics of primary extranodal non-Hodgkin’s lymphoma (pENL) patients, focusing on treatment and survival outcome. Materials and Methods: Between October 2003 and March 2012, 802 patients with non-Hodgkin’s lymphoma (NHL) were diagnosed and treated in two different cancer centers of Southern Turkey. Results: pENL, constituted 12.4% (100/802) of all NHL studied during this period. Median age of the patients was 56 years (range 17-87 years) and the male: female distribution was 3:2. Eighty-five of 100 patients (85%) were in stage I/II, 9/100 (9%) in stage III, whereas 6/100 (6%) were in stage IV. Head and neck constituted the most common site (51/100, 51%), followed by gastrointestinal tract (GIL) (37/100, 37%), and cerebrum (CL) (5/100, 5%). Diffuse large B cell lymphoma (DLBCL) was the most common histological type, observed in 53% of patients, followed by marginal zone extranodal lymphoma (13%). Most of patients (76%) received a CHOP containing regimen. Complete remission (CR) were achieved in 71% of patients. The median follow-up duration of all patients was reported as 37.6 months (range, 0.8-165 months). This period was reported as 137.5 months (range, 117.5-1578.6 months) in gastrointestinal lymphoma (GIL) patients, 119.0 months (range, 91.8-146.1 months) in headand neck lymphoma (HNL) patients, and 18.4 months (range, 12.6-24.1 months) in cerebral lymphoma (CL) patients. Conclusions: Head and neck, and the gastrointestinal tract were the two most common extranodal sites observed. Histologically DLBC accounted for the majority of cases. Most patients were on earlier stages, had low-low intermediate IPI scores and had a favorable prognosis.     

Non-Hodgkin's lymphomas in Turkey

Between 1982 and 1986, 185 adult patients with non-Hodgkin's lymphoma (NHL) were studied at Cukurova University Hospital (CUH), Adana, Turkey. This constituted 13% of all malignant neoplasms diagnosed in the oncology clinic. The mean age for men was 45.5 years and 41 years for women. Fifty-four percent of the cases were nodal lymphoma and the remaining 46% were extranodal lymphoma. Sixty-six percent of the extranodal lymphoma cases were gastrointestinal lymphoma. The stomach was the most common localization (43%), followed by intestinal involvement (30%) and abdominal mass (27%). The mean age of the patients with gastric lymphoma was 49 years, and 36 years for patients with abdominal mass. The following conclusions were reached: (1) the relative frequency rate of NHL in South Turkey is higher than Turkey averages, but similar to other Middle East countries; (2) extranodal lymphomas account for more than 40% of all lymphomas; (3) gastric lymphoma is more prevalent than intestinal lymphoma in our region; and (4) compared with western gastric lymphomas, our patients were 10 years younger and had a higher incidence of small lymphocytic and immunoblastic lymphoma.     

Extranodal lymphomas in the public health system in Chile: Analysis of 1251 patients from the National Adult Cancer Program

The aim of the study was to describe the clinical and epidemiological characteristics, anatomic and histologic distribution, and treatment results of extranodal lymphomas (ENLs), diagnosed and treated in the public health system in Chile. We included patients with ENL diagnosed from 1998 to 2014, in 17 cancer centers, registered prospectively in the database of the National Adult Cancer Program (PANDA) of the Ministry of Health. Treatment was based on the local protocols for each lymphoma subtype. Extranodal lymphoma was documented in 1215 of 4907 non‐Hodgkin lymphomas diagnosed in that period (25%). Median age was 59 years (range, 16‐95), and 55% were female. The gastrointestinal (GI) tract was the most common location (38%), followed by the head and neck (24%) and the skin (15%). B‐cell lymphomas accounted for 78% of cases, diffuse large B‐cell lymphoma being the most common histologic subtype (68%). Mycosis fungoides/Sezary syndrome was the most frequent T‐cell subtype (36%), followed by NK/T‐cell lymphomanasal type (24%). In comparison with western countries, Chile showed a significantly high prevalence of NK/T‐cell lymphoma nasal type, while the frequency of B‐cell ENL and the anatomic distribution appeared similar, being GI the most commonly involved site.     

Primary extranodal and nodal non-Hodgkin's lymphoma: A survey of a population-based registry

In a population-based registry, there were 580 patients with non-Hodgkin's lymphoma (NHL); 236 had primary extranodal lymphoma (41%). The initial localization of the primary extranodal lymphomas varied markedly, although 36% were primary gastrointestinal lymphomas. Histological classification was performed by a regional panel of pathologists according to the Kiel Classification and the International Working Formulation. Twelve per cent of the patients with nodal NHL had a localized disease in contrast to 40% with primary extranodal NHL. Low grade lymphomas were encountered in 30 and 10% of the patients with primary nodal and extranodal NHL, respectively. Recurrence-free survival rate for patients with localized low-grade malignancy and disseminated intermediate grade NHL is significantly better for extranodal lymphoma than for nodal NHL. Patients with disseminated high-grade extranodal NHL had the worst prognosis of all. We conclude that primary nodal and primary extranodal lymphomas should be considered as distinctive and separate entities.     

Patterns of disease in localized extranodal lymphomas

The site of origin of lymphoid tissue is an important determinant of lymphocyte migration patterns. The association of gastrointestinal (GI) and Waldeyer's ring lymphoma and the unique lymphocyte migration pattern of gut-associated lymphoid tissue (GALT) have been previously described. To establish whether predictive clinical patterns of disease occur in localized Non-Hodgkin's lymphoma, survival and relapse patterns for 496 patients with stage I and II non-Hodgkin's lymphoma (NHL) treated with loco-regional irradiation (XRT) alone were examined. We identified 139 patients with GALT lymphoma (defined as arising from primitive gut and including Waldeyers' ring, thyroid, and GI lymphomas) and 87 patients with extranodal non-gut-associated lymphoma (ENL). Survival and relapse data were assessed in multifactorial analysis to correct for previously identified other prognostic variables. GALT lymphomas (GALT-L) have a survival advantage compared with other ENL (P = .017) independent of stage and histology. A difference in distant relapse (DR) rate between GALT-L and other ENL (P = .0002) was also identified. The presentation site of localized extranodal NHL is predictive of clinical behavior and is an independent determinant of outcome. This may be an expression of lymphocytic origin and determinants of migration patterns.     

青海地区淋巴瘤253例临床特点与预后因素分析

目的 分析淋巴瘤的临床特点及预后相关因素.方法 对青海地区253例淋巴瘤患者临床资料进行回顾性研究并随访.采用Kaplan-Meier法分析患者不同临床特点及实验室检查的生存期差异.采用Cox比例风险回归模型分析影响预后的因素.结果 253例淋巴瘤患者中,男、女比例为1.56∶1,中位年龄为48岁,发病年龄高峰为40岁和60岁左右.结内起病（56.13％）较多,结外起病常见部位为鼻咽部和胃肠道.病理类型为霍奇金淋巴瘤40例（15.81％）,非霍奇金淋巴瘤213例（84.19％）.前者以结节硬化型（72.50％）最多见;后者中B细胞淋巴瘤148例（69.48％）,最常见的病理类型为弥漫大B细胞淋巴瘤（39.91％）、滤泡细胞淋巴瘤（12.21％）及周围T细胞非特殊型淋巴瘤（9.39％）.单因素预后分析显示,临床分期、有无B症状、血红蛋白（Hb）、乳酸脱氢酶（LDH）、国际预后指数（IPI）及治疗方案与非霍奇金淋巴瘤预后密切相关（均P＜0.05）.Cox回归模型多因素分析显示,临床分期、IPI及治疗方案与非霍奇金淋巴瘤的预后关系密切,差异有统计学意义（均P＜0.05）.结论 青海地区淋巴瘤发病年龄有两个高峰,结内发病高于结外,B细胞淋巴瘤多见,IPI分组适用于该地区非霍奇金淋巴瘤的预后评估,并可作为其独立的预后因素.     

Oral Extranodal Non Hodgkin’s Lymphoma: Series of Forty Two Cases in Malaysia

Background: Lymphoma is a malignant neoplasm of lymphoid tissue classified into Hodgkin’s and non-Hodgkin’s types. It mostly affects lymph nodes although a considerable proportion of Non-Hodgkin’s casesoccur in extranodal sites. Materials and Methods: Selected cases diagnosed as non-Hodgkin’s lymphoma (NHL)during the period of 1980 to 2012 were retrieved from the archives of the Oral Pathology Diagnostic Laboratory,Faculty of Dentistry, University of Malaya. The sections from the formalin-fixed paraffin embedded tissue blockswere stained with H&E as well as with LCA, CD20, and CD3. Results: The mean age was 41.6 years with amale: female ratio of 1.3:1. Out of the forty two cases, nineteen were Malays, eighteen were Chinese, followedby Indians (3) and Indonesians (2). The most common site of involvement was the mandible (22.2%), followedby the maxilla and palate (19.4% each). Most of the lesions presented as a painless progressive swelling. Onlythirty six cases were further subdivided into B or T cell types. The majority were B-cell type (26 cases), of these 6cases were Burkitt’s lymphomas. Only ten cases were T-cell lymphoma, with three cases of NK/T-cell lymphoma.Conclusions: In this series of 42 patients diagnosed as extranodal non-Hodgkin’s lymphoma, the lesions appearedas painless swellings, mostly in men with the mandible as the most frequent site of involvement. Majority wereB-cell lymphomas with Malays and Chinese being equally affected whereas lymphomas were rare in the Indianethnicity. T-cell lymphomas were found to be common in the Chinese ethnic group.     

原发性结外非霍奇金淋巴瘤127例临床特征及误诊分析

目的分析淋巴结外非霍奇金淋巴瘤(NHL)的临床表现特点及误诊原因。方法回顾性分析127例原发结外非霍奇金淋巴瘤的发病情况、原发部位分布情况、首发症状及误诊情况等。结果原发于结外的非霍奇金淋巴瘤12／例，占同期非霍奇金淋巴瘤53．81％(127／236)；原发部位前5位依次为：胃肠道21．26％(27／127)，鼻腔18．9％(24／127)，Waldeyer环14．96％(19／127)，脾脏12．6％(16／127)，皮肤6．3％(8／127)；结外奇非霍金淋巴瘤误诊率为69．29％(88／127)；结外非霍奇金淋巴瘤首发症状因原发部位不同而临床表现各异，无特异性。结论结外NHL发生率较高，其临床表现缺乏特异性，早期易误诊。     

Prognostic Factors in Non-Hodgkin Lymphoma Stage I Treated with Radiotherapy

The results of treatment in 175 consecutive patients with non-Hodgkin lymphoma (NHL) clinical stage I treated between 1969 and 1984 were analysed according to different pretreatment prognostic variables. Treatment consisted of radiotherapy in 166 of the 175 patients. The estimated 5 and 10-year disease-free survival rates (DFS) were 63 % and 60% and the survival rates at 5 and 10 years 82% and 76% respectively. Lymphomas arising from gut-associated lymphoid tissue, i.e. Waldeyer's ring, the thyroid and the gastrointestinal tract had a more favourable clinical course (10-year projected DFS 83%) than nodal (50%) and other extranodal lymphomas. Although the number of patients with other extranodal sites was small, sites such as testis, nasal cavity, paranasal sinus and extradural space seemed to have a high relapse rate. Unfavourable clinical courses were also observed among nodal high-grade NHL if the lymph nodes were larger than 5 cm in diameter. Chemotherapy before radiotherapy may be recommended in NHL subgroups with a high relapse rate and which today are potentially curable with chemotherapy, i.e. high-grade NHL. This study indicates that large nodal lymphomas and some extranodal sites belong to this group.     

Features of primary extranodal lymphoma in Kanagawa, a human T-cell leukemia virus type 1 nonendemic area in Japan

We sought to determine the frequency of primary extranodal lymphoma (ENL) and its characteristics in Kanagawa, a human T-cell leukemia virus type 1 (HTLV-1) nonendemic area in Japan. Subjects were 847 newly diagnosed patients with malignant lymphoma at the Yokohama City University Hospital and 8 affiliated hospitals mainly located in Kanagawa prefecture from 1999 to 2005. We compared the clinicopathological characteristics of primary ENL with primary nodal lymphoma (NL). Histological specimens were evaluated according to the World Health Organization classifications. A total of 395 (46.6%) and 452 (53.4%) patients had primary ENL and primary NL, respectively. The frequency of primary ENL increased with age. Primary extranodal sites included the gastrointestinal tract (30.4%), Waldeyer’s ring (17.8%), orbits (7.0%), soft tissue and subcutaneous tissue (5.2%), bone (4.6%), skin (4.3%), thyroid gland (4.3%), testis and prostate (3.3%), bone marrow (3.3%), nasal and paranasal cavities (2.6%), salivary glands (2.3%), lung and pleura (2.0%), breast (1.8%), central nervous system (1.0%), uterus and ovary (0.5%), and others (9.8%). Among the 395 cases of primary ENL, diffuse large B-cell lymphoma (61.2%) was most frequently diagnosed, followed by extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (13.3%) and follicular lymphoma (5.6%). The frequency of primary ENL is approximately 50% of the total lymphoma cases in Kanagawa, an HTLV-1 nonendemic area in Japan. This frequency appears to be higher than that in Western countries.     

Non-Hodgkin's lymphomas in the Middle East. A study of 417 patients with emphasis on special features

A total of 417 evaluable patients with non-Hodgkin's lymphomas were diagnosed between January 1974 and December 1983 at the American University of Beirut Medical Center in Beirut, Lebanon. Of these, 179 (43%) patients had nodal lymphomas, and 183 (44%) had extranodal lymphomas. The commonest lymphoma was diffuse large cell (27%), followed by large cell immunoblastic (21%). The histopathologic pattern was follicular in 18% of the nodal lymphomas and in 5.3% of the extranodal forms. The most common site of extranodal lymphoma was the gastrointestinal tract (46.5%), followed by Waldeyer's ring (19%). Small intestinal lymphomas were three times more common than gastric lymphomas. Immunoproliferative small intestinal disease (IPSID) was diagnosed in 20 of 59 patients who had primary small intestinal lymphoma. Of the 34 patients who had Waldeyer's ring lymphoma, 7 had gastrointestinal involvement at some time during the course of the disease. Nodal lymphomas were associated with poor prognostic factors: 82% were diffuse; 77% had advanced disease at presentation; 77% had intermediate- or high-grade malignancy lymphoma; 40% had marrow involvement; and 46% had B symptoms. In children, the most common lymphoma was Burkitt's, and 80% of pediatric lymphomas were high-grade malignancy. In conclusion, this study delineates the special features of non-Hodgkin's lymphomas in the Middle East: The presence of IPSID; the high incidence of extranodal forms, in particular the intestinal ones; and the rarity of follicular lymphomas.     

我国淋巴结外淋巴瘤的临床特点

目的：了解中国人的结外淋巴瘤的临床特点。方法：将解放军总医院病理科自１９５５ 年至１９９７ 年４２ 年间诊断为淋巴瘤的１６３１ 例作为分析对象。从患者的性别、年龄、部位、病理诊断共４ 个方面进行统计分析。结果：１６３１例淋巴瘤中, 结内６８８ 例, 占４２－１８ ％ ; 结外９４３ 例, 占５７－８２ ％ 。在９４３ 例结外淋巴瘤中, 见于消化道２２９ 例, 占２４－２８％ ;鼻腔１２１ 例,占１２－８３ ％ ;皮肤１０４ 例,占１１－０３ ％ ;扁桃体８４ 例,占８－９ ％ ;口腔７７ 例,占８－１７％ ;骨５５ 例,占５－８３ ％ ;鼻咽部４０ 例,占４－２４ ％ 。其余部位为软组织３７ 例,腹腔２６ 例,涎腺２４ 例,肝脏２２ 例,眼部１９ 例,肺１９ 例,颅内１４ 例,睾丸１１ 例,脾１０ 例,纵隔９ 例,喉部８ 例,甲状腺６ 例,胸腔６ 例,乳腺５ 例,胰腺及膀胱各２ 例,肾上腺、肾及卵巢各１ 例。结论：１ 本组材料中结外淋巴瘤高达５８ ％ 。超过结内淋巴瘤的比率,这是到目前为止文献报道中比例最高的。这是否是中国人淋巴瘤的特点,还有待于今后进一步证实;２ 美国白种人的结外淋巴瘤好发部位是胃、皮肤、扁桃体、涎腺、骨,而本组结果得出的结外淋巴瘤的好发部位依次     

Diagnostic and Prognostic Relevance of Bone Marrow Microenvironment Components in Non Hodgkin’s Lymphoma Cases Before and After Therapy

Objective: To evaluate stromal cells of the bone marrow microenvironment (BMM) in bone marrow trephine biopsy (BMTB) specimens, with a focus on fibronectin, tumor necrosis factor- alpha (TNF-α) and L-selectin in Non- Hodgkin’s lymphoma (NHL) patients, before and after therapy. Materials and Methods: A total of 80 de novo NHL patients, 64 with B-cell lymphomas 80% , (follicular cell lymphoma (FCL) in 32, chronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL) in 12, and diffuse large cell lymphoma in 20) and 16 with T-cell lymphomas (20%) all diagnosed as T-Lymphoblastic lymphomas, were evaluated before and after therapy. For comparison, 25 age and sex matched BM donors, were included as a control group. BMTB material and BM aspirates were taken for morphological assessment of stromal cells, the plasma of these samples being examined for TNFα and L-selectin by ELISA, and fibronectin by radial immunodiffusion (RID). Results: BM stromal cells comprising reticular macrophages and fibroblasts were elevated in 53.3% of NHL cases at diagnosis, while BM fibronectin levels were decreased and BM TNFα and L-selectin were higher than in controls (p<0.05). In NHL cases, elevated values of BM TNFα and BM L-selectin were associated with signs of aggressive disease, including >1 extra nodal sites, detectable B symptoms, high grade, BM and CNS invasion, and a high International prognostic index (IPI) (p<0.05). Conclusion: BMM components, TNFα, L-selectin and fibronectin, in NHL can be useful in evaluating disease activity, extent and response to treatment and as prognostic markers according to the IPI.     

Diffuse Large B Cell Lymphoma (DLBCL) in Pakistan: An Emerging Epidemic?

There has been a recent concern among oncological clinicians and pathologists of our region regarding the ‍disproportionate increase in the number of patients presenting with diffuse large B cell lymphoma (DLBCL). This ‍prompted us to conduct a thorough, hospital-based epidemiological study in a major referral center of Pakistan. A ‍total of 780 specimens were collected over last half decade from cases classified as adult Non Hodgkin’s lymphoma ‍(NHL). Out of these 780, 596 (76.4 %) were diagnosed as DLBCLs. The gender ratio was 2.3: 1 (M: F) and the ‍median age was 47.2 years, with an age range of 15-85 years. Nodal-NHLs constituted 42.2 % of all adult NHLs, with ‍the cervical lymph nodes as the most frequent nodal site of presentation. The most frequent extranodal site was the ‍gastro-intestinal tract (GIT), followed by the head and neck. In conclusion, we document an astonishingly high ‍number of DLBCL amounting to an emerging epidemic in Pakistan, with a consideration of probable etiopathogenetic ‍factors. ‍     

A PCR analysis of bcl-2 gene rearrangement in nodal and extranodal non-hodgkins-lymphoma (nhl) - some unusual observations in saudi patients

Non-Hodgkin's lymphoma (NHL) is the most common malignancy referred to our institute which is the largest tertiary referral cancer centre in Saudi Arabia, The proportion of follicular low grade NHL appears to be extremely small in this population (<5% of all NHL). To date, there is no data available regarding any correlation between bcl-2 gene rearrangement and different cell types of nodal and extranodal NHLs in Saudi patients. We used a sequential polymerase chain reaction (PCR) technique to determine the frequency of bcl-2/J(H) recombination occurring via the major breakpoint region (mbr) in 16 GI tract NHLs including 4 MALT lymphomas and 13 follicular (nodal) NHLs. The results showed only 2/13 (15%) nodal follicular NHLs with bcl-2/J(H) fusion DNA whereas 9/16 (56%) of the extranodal NHLs with at least 2 of them exhibiting MALT characteristics were positive for the bcl-2 gene rearrangement. A breakdown of the proportion of extranodal NHLs of different cell types showing bcl-2 rearrangement via mbr was as follows: 5/8 diffuse large non-cleaved cell (DLNCC), 1/3 diffuse small cleaved cell (DSCC), 1/1 follicular small cleaved cell (FSCC) and 2/4 MALTs. The PCR amplified bcl-2/J(H) fusion DNA from 5 randomly selected tumors (2 MALTs, 1 DLNCC, 1 DSCC and 1 nodal follicular lymphoma) were cloned and sequenced. All 5 of them showed different bcl-2/J(H) N-regions confirming the clonality of each tumor sample. The data indicating a very low incidence of bcl-2 translocation in nodal follicular NHLs and a surprisingly high incidence of it in extranodal NHLs are intriguing, and quite contrary to the findings in Western patients. These unusual observations warrant further studies and may suggest that different genetic events are involved in the development of extranodal NHLs including MALT and follicular center-cell NHLs in Saudi patients.     

Primary extranodal non-Hodgkin's lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry.

BACKGROUND: The definition of primary extranodal non-Hodgkin's lymphoma (NHL) is a controversial issue, especially in patients where both nodal and extranodal sites are involved. PATIENTS AND METHODS: The impact of different definitions of primary extranodal NHL on incidence and prognosis is explored using data from a population-based NHL registry. RESULTS: Using liberal criteria, 389 (34%) cases were classified as primary extranodal NHL. Overall survival (OS) rates of nodal and extranodal NHL patients defined this way were comparable; however, extranodal NHL patients had a better disease-free survival (DFS). When strict criteria were applied, 231 cases (20%) were classified as primary extranodal NHL. OS and DFS rates of extranodal NHL patients defined this way were superior to nodal NHL patients; however, the difference in OS was reversed after correction for differences in International Prognostic Index and malignancy grade. CONCLUSION: This study illustrates the selection bias that is introduced when a strict definition of primary extranodal NHL, that excludes cases with disseminated disease, is used. Patients with primary extranodal NHL were found to have a superior DFS, irrespective of which definition of primary extranodal NHL was used.     

国际淋巴瘤放射治疗协作组(ILROG)现代放射治疗照射野与剂量指南——结外淋巴瘤(ENL)

ENL约占NHL总数的1/3。放疗常用于初始治疗(尤其是惰性结外淋巴瘤)、系统治疗后的巩固治疗、挽救性治疗以及姑息治疗。由于ENL临床表现多种多样,可以累及全身各器官,病理亚型丰富,给常规临床诊疗以及开展前瞻性和回顾性研究带来诸多困难。不同中心或医生使用的放疗方法亦不尽相同。迄今,ENL的放疗缺乏相关指南。本文中ILROG专家们统一了ENL治疗原则,同时阐述了常见ENL的模拟定位、靶区定义和计划制定,并详尽介绍了放疗靶区勾画方法。本文应用的ISRT新原则,与之前发表的HL和结内NHL指南相同。本文靶区基于ICRU文件,与实体肿瘤相同。此外,本文根据组织分型、解剖结构、治疗目的以及放疗前可能应用的其他治疗模式,为不同器官淋巴瘤放疗给予特殊推荐。     

Expression of inhibitor of apoptosis proteins in B-cell non-Hodgkin and Hodgkin lymphomas

BACKGROUND: Inhibitor of apoptosis proteins (IAPs) inhibit apoptosis by binding specific caspases, and possibly by other mechanisms. Eight IAPs have been identified in humans, of which cIAP1, cIAP2, and XIAP are well known. IAPs are being investigated as potential treatment targets in cancer patients. METHODS: cIAP1, cIAP2, and XIAP were assessed in lymphoma cell lines, 240 B-cell non-Hodgkin lymphoma (NHL) tumors, and 40 Hodgkin lymphoma (HL) tumors. RESULTS: All IAPs were expressed in most NHL and all HL cell lines. In NHL tumors, cIAP1 was expressed in 174 (73%), cIAP2 in 115 (48%), and XIAP in 37 (15%). cIAP1 was positive in all precursor B-cell lymphoblastic lymphoma/leukemia (LBL) and nodal marginal zone B-cell lymphoma (MZL), over 90% of follicular lymphoma and diffuse large B-cell lymphoma (DLBCL), and approximately 50% to 60% of myeloma, Burkitt lymphoma (BL), lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (LPL/WM), small lymphocytic lymphoma/ chronic lymphocytic leukemia (SLL/CLL), extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT-lymphoma), splenic MZL, and mantle cell lymphoma. cIAP2 was positive in all MALT-lymphoma, over 90% of precursor B-cell LBL (94%), most BL (75%), LPL/WM (71%), and SLL/CLL (67%), and approximately 40% to 60% of follicular lymphoma, myeloma, and DLBCL. XIAP was positive most cases of precursor B-cell LBL (57%) and approximately 30% to 40% of nodal MZL, BL, and DLBCL. In HL tumors, cIAP1 was positive in 30 (75%), cIAP2 in 27 (68%), and XIAP in 23 (58%), and did not correlate with histologic type. CONCLUSIONS: Differential expression of IAPs in B-cell lymphomas suggests differences in pathogenesis that may have implications for novel treatment strategies targeting IAPs.     

非霍奇金淋巴瘤1012例临床病理分析

目的:了解我院近10年来非霍奇金淋巴瘤(NHL)的发病特点,分析影响NHL预后的相关因素.方法:回顾性分析了近10年来我院收治的1012例NHL患者的临床病理特点,对影响新疆地区NHL生存率及预后的临床病理因素进行分析.结果:1012例NHL中以40～60岁汉族男性发病多见,最主要病理类型依次为弥漫性大B细胞淋巴瘤(DLBCL)346例(34.1%),外周T细胞淋巴瘤(PTCL)185例(18.3%),滤泡淋巴瘤(FL)97例(9.6%),黏膜相关淋巴组织淋巴瘤(MALT)94例(9.3%),NK/T细胞淋巴瘤62例(6.1%),T-淋巴母细胞淋巴瘤(T-LBL)47例(4.6%).结性起病的淋巴瘤619例(61.2%),结外起病的淋巴瘤393例(38.8%).本组维吾尔族女性FL淋巴瘤患者人数比例较汉族女性患者高(P=0.002),汉族男性PTCL患者人数比例高于维吾尔族男性患者(P=0.015).5年总生存率为45.8%.单因素分析显示临床分期,行为状态评分(PS),B症状,年龄,肿块大小,血清乳酸脱氢酶(LDH),结外器官受侵数目及IPI是NHL的预后因素(P＜0.05).多因素分析提示T细胞来源,Ⅲ～Ⅳ临床分期,IPI评分3～5分及LDH增高是NHL独立的预后不良因素(P＜0.05).结论:新疆地区NHL发病以中年多见,结性起病者多于结外起病,B细胞淋巴瘤多于T细胞淋巴瘤.免疫分型、临床分期、IPI、血清LDH水平与NHL预后相关.     

Clinico-pathological features of malignant lymphomas in 294 Hong Kong Chinese patients, retrospective study covering an eight-year period

The clinical records and histological material from 294 adult Chinese patients with malignant lymphoma were examined. These patients were first seen at the Queen Mary Hospital, Hong Kong, during the 8-year period 1975-82. There were 27 patients (9.2%) with Hodgkin's disease (HD) and 267 with non-Hodgkin's lymphoma (NHL). The median age at presentation was younger for HD (45 years) and the male: female ratio was higher (2:1) than the corresponding figures for NHL of 51 years and 1.4:1. In 76 patients (28.5% of NHL), the disease was thought to have originated in an extra-nodal site, 48 of these cases being gastrointestinal lymphomas. It was possible to reclassify 234 NHL according to the Rappaport and Kiel classifications, and the Working Formulation (WF) proposed by the US National Cancer Institute Study; for HD, the Rye classification was used in 26 cases where suitable material was available. Nodular/follicular lymphomas made up 17.1% of nodal NHL and 5.3% of extra-nodal NHL. The "histiocytic" (Rappaport) or large-cell (WF) subtype was the commonest amongst diffuse NHL. There were only four cases of Burkitt's lymphoma. For HD, the nodular sclerosing subtype was commonest in females (5 out of 8 cases) and for males, the commonest was mixed cellularity (10 out of 18 cases). Of patients with nodal NHL 64.7%, presented with Stage IV disease. For HD, there were about equal numbers of patients presenting with Stage II and Stage IV disease (10 and 9 respectively). The low incidence of Hodgkin's disease and of follicular lymphomas is comparable to figures from other "oriental" countries such as Japan.