Primary Hepatic Low-Grade B-Cell Lymphoma of the Mucosa-Associated Lymphoid Tissue Type: A Case Report and Review of the Literature

Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis A presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin D1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.     

Sustained Remissions with Long-term Weekly Chlorambucil Maintenance Therapy in Patients with Mucosa-Associated Lymphoid Tissue Lymphoma

Five patients with mucosa-associated lymphoid tissue (MALT) lymphoma (Helicobacter pylori negative) were initially treated with daily chlorambucil (Chl, 0.06-0.08 mg/kg per day) and achieved complete remission (n = 4) or partial remission (n = 1).They were then given long-term weekly Chl as maintenance therapy with good results. One patient came off treatment and has remained in continued remission for 44 months. This simple, low-cost, and convenient approach deserves further evaluation in patients with MALT lymphoma and other indolent and incurable lymphoproliferative disorders.     

Absence of Chlamydial Infection in Japanese Patients with Ocular Adnexal Lymphoma of Mucosa-Associated Lymphoid Tissue

Ocular adnexal extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (ocular adnexal MALT lymphoma) are predominately low-grade, small B-cell types and may be caused by several putative inflammatory agents. Twenty-three ocular adnexal MALT lymphoma cases, the monoclonality of which was confirmed by examination of immunoglobulin heavy chain gene rearrangement and/or cell surface antigens, were analyzed for evidence of several causative factors. A serologic evaluation of our series of patients showed no evidence of infection by Epstein-Barr virus, hepatitis C virus, or Chlamydophila psittaci. Two patients tested positive for serum antibodies for autoimmunity, and another 2 patients tested positive for antibodies against Chlamydia trachomatis. Polymerase chain reaction analysis did not reveal the presence of the chlamydial 16S ribosomal RNA (rRNA) gene or the 16S-23S spacer rRNA gene. These results indicate that the inflammatory agents in our series of ocular adnexal MALT lymphomas are still unknown and that some types of chlamydial infections are not associated with orbital adnexal MALT lymphoma in southern regions of Japan.     

Synchronous Adenocarcinoma and Mucosa-associated Lymphoid Tissue Lymphoma of the Colon

Mucosa-associated lymphoid tissue (MALT) tumors are a distinct subtype of non-Hodgkin’s lymphoma. Synchronous appearance of adenocarcinoma and colonic MALT lymphoma in the same patient is quite rare. In the present report, we describe a 68-year-old female who presented with a history of bleeding per rectum. She had no history of fever, loss of weight or drenching night sweats. Rectal examination revealed no abnormality. Colonoscopy showed a large ulceroproliferative mass arising from the hepatic flexure, biopsy of which came out to be adenocarcinoma of colon. A right hemicolectomy was performed and microscopic study revealed the tumor type to be synchronous adenocarcinoma with lymphoma. The final diagnosis of this patient turned out to be a synchronous manifestation of both colonic adenocarcinoma and colonic MALT lymphoma. Although the patient remains asymptomatic two years after surgery, the case highlights the therapeutic dilemma that prevails in the definitive management in such scenarios.     

The Microvacular Thrombi of Colonic Tissue in Ulcerative Colitis

Mucosal microvacular thrombi in rectal biopsies were observed in some ulcerative colitis (UC). Heparin may be effective in steroid resistant UC in some studies, however, the new results of meta-analysis demonstrated a non-significant effect of heparin in controlled clinical trials, differing markedly from observational studies. The objective of this study was to identify colonic microvascular thrombi in larger cases with UC, and analyse its possible risk factors: age, gender, histologic score, extent of lesions and operation or biopsy specimens, and assess the significance of microvascular thrombosis in patients with UC. The microvascular thrombi were identified by immunohistochemical staining with anti-CD61 monoclonal antibody and Martius scarlet blue (MSB) staining in 40 colonic tissue samples of UC (31 biopsy specimens and nine operated cases) and 12 cases of normal colon tissue from operated colonic carcinoma. Logistic regression analysis was used to assess the relationship of age, gender, degree of histology, origin of the specimens, extent of lesions and microvascular thrombi examined. Microvascular thrombi were positive in 14 of 40 UC cases, and none in the controls. The presence of microvascular thrombi was related to operation specimens with odds ratio 11.667, P=0.0179, it might be also related to histologic score (OR=1.350) and extent of lesions (OR=1.619). These results suggest that microvascular thrombosis may be one of the important pathogenesis in some UC, and that the effect of anticoagulant treatment still needs to be assessed.     

溃疡性结肠炎的并发症

溃疡性结肠炎是慢性复发性疾病,其并发症直接影响该病的预后。本文以1993年全国慢性非感染肠道疾病学术研讨对本病会制定的诊断标准,对北京协和医院1974年1月至1995年1月溃疡性结肠炎住院和门诊病人483例进行了分析,着重探讨并发症的种类与疾病严重程度的关系以及对预后的影响。本组病人并发症的发生率为6.2％(30/483)。重症患者并发症的发生率为24.3％。并发症的种类,按其发生频率的多少依次排列如下:严重电解质紊乱(9.01％)、肠梗阻(8.11％)、人出血、菌群失调,肠穿孔(各3.60％)、败血症(2.70％)、肠瘘、癌变(各1.80％).中毒性肠扩张(0.90％)。本组溃疡性结肠炎早期并发症,除电解质紊乱外尚有大出血、中毒性肠扩张、败血症多见,随病程迁延则以肠梗阻、肠瘘、肠穿孔和癌变为主。并发症多发生于重度活动期(24.3％);其中病变累及全结肠为82％,左半结肠仅为18％。住院病人中该病病死率为6.08％,并发症组为23.08％,高于无并发症组(2％)。因此,对溃疡性结肠炎的并发症,要提请广大临床医师注意:需早期诊断,积极治疗,以减少病死率。     

溃疡性结肠炎的并发症

目的:探讨溃疡性结肠炎并发症的种类与疾病严重程度的关系以及对预后的影响.方法:以1993年全国慢性非感染肠道疾病学术研讨会制定的对本病的诊断标准,分析了北京协和医院1974年1月至1995年1月溃疡性结肠炎住院和门诊病人483例.结果:本组病人并发症的发生率为6.2%(30/483).重症患者并发症的发生率为24.3%.并发症的种类按其发生频率的多少依次为:严重的电解质紊乱(9.01%)、肠梗阻(8.11%)、大出血、菌群失调、肠穿孔(各3.6%)、败血症(2.70%)、肠瘘、癌变(各1.80%)、中毒性肠扩张(0.90%).本组溃疡性结肠炎早期并发症,除电解质紊乱外尚有大出血、中毒性肠扩张、败血症多见,随病程迁延则以肠梗阻、肠瘘、肠穿孔和癌变为主.并发症多发生于重度活动期(24.3%);其中病变累及全结肠为82%,左半结肠仅为18%.住院病人中该病病死率为6.08%,并发症组为23.08%,高于无并发症组(2%).结论:溃疡性结肠炎的并发症需早期诊断,积极治疗以减少病死率.     

Intestinal Mucosa-Associated Microflora in Ulcerative Colitis Patients Before and After Restorative Proctocolectomy with an Ileoanal Pouch

Purpose  This study was designed to identify the mucosa-associated microflora in patients with severe ulcerative colitis before and after restorative proctocolectomy with ileoanal pouch construction in comparison with historic controls. Methods  Ten patients with a diagnosis of ulcerative colitis were evaluated. Mucus was collected during colonoscopy from all segments of the colon and terminal ileum before surgery, and from the ileal pouch two and eight months after ileostomy closure. The prevalence and mean concentration of the mucosa-associated microflora were compared over time and with historic controls. Results   Veillonella sp was the most prevalent bacterium in patients and controls. Klebsiella sp was significantly more prevalent in the ileum of controls, was not found in patients with ulcerative colitis, and after proctocolectomy returned to values found in controls. Some bacteria such as Enterobacter sp, Staphylococcus sp (coag-), Bacteroides sp (npg), Lactobacillus sp, and Veillonella sp had higher mean concentrations in the ileal pouch of patients after surgery than in controls. Conclusion  No bacterium was identified that could be exclusively responsible for the maintenance of the inflammatory process. The mucosa-associated microflora of patients with ulcerative colitis underwent significant changes after proctocolectomy with ileal pouch construction and returned to almost normal values for some bacteria. Presented at the meeting of the International Society of University Colon & Rectal Surgeons, Budapest, Hungary, June 6 to 10, 2004.     

Primary Pulmonary Mucosa-associated Lymphoid Tissue Lymphoma with the High Expression of IgG4

This is the first report describing primary pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma with the high expression of IgG4. The histological findings were compatible with the diagnostic criteria for MALT lymphoma and IgG4-related respiratory disease (IgG4-RRD). An unfixed sample for Southern blotting was not obtained since computed tomography findings showed multiple lung cysts, which is rare in patients with MALT lymphoma. However, polymerase chain reaction using paraffin sections showed the clonality of the immunoglobulin heavy chain variable region gene rearrangement, confirming a diagnosis of MALT lymphoma. This is an instructive case in which primary pulmonary MALT lymphoma was histologically compatible with IgG4-RRD.     

重症溃疡性结肠炎的内科治疗

参照1973年全国慢性非感染肠道疾病学术研讨会制定的溃疡性结肠炎的诊断标准,对北京协和医院1974年1月至1995年1月的溃疡性结肠炎住院病人共148例进行了分析,着重探讨了我院对重症溃疡性结肠炎的药物治疗经验。结果显示:21年间溃疡性结肠炎在内科消化病的年住院率呈上升趋势,重症患者占 72.3 ％。其临床治疗仍以激素,水杨酸偶氮磺胺吡啶和免疫抑制剂为主要治疗药物。本病在我院内科治疗的临床缓解率达95.9 ％,其中重症的临床缓解率达95,3 ％,死亡率为6.08 ％。我们提出对溃疡性结肠炎的内科治疗应遵循尽早控制症状、维持缓解、预防复发、防治并发症和掌握手术时机的原则;并根据病变的范围、疾病的活动性和严重程度、病程、病人的全身情况、以前用药情况和有无并发症等进行综合治疗。     

Presence of a high-grade component in gastric mucosa-associated lymphoid tissue (MALT) lymphoma is not associated with an adverse prognosis

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma and diffuse large B cell lymphoma (DLBCL) show a spectrum of disease characterized by varying proportions of low-grade and high-grade components. While the natural history and optimum treatment for low-grade gastric MALT lymphoma and DLBCL is well established, the prognosis and optimal treatment of patients with both low- and high-grade components is not well established. The purpose of our study was to evaluate the clinical characteristics, survival outcomes, and prognostic factors of patients with gastric MALT lymphoma and gastric DLBCL. A retrospective review of patients with gastric MALT lymphoma, gastric DLBCL, or MALT lymphoma with a high-grade component treated at our centers from 1994 to 2006 was performed. Patients were divided into three categories: “pure MALT lymphoma,” “MALT lymphoma with high-grade component” (mixed), and “pure DLBCL.” Seventy-six patients were included in our study—26 with pure MALT, 22 with MALT with high-grade component (“mixed”), and 28 with pure DLBCL. Pure MALT lymphoma and mixed lymphoma patients had similar clinical characteristics, whereas pure DLBCL patients had less favorable disease characteristics with significantly poorer performance status, higher number of extranodal sites of disease, higher stage, and larger proportion of bone marrow involvement and international prognostic index (IPI) scores compared with mixed lymphoma. The majority of mixed lymphoma (72.7%) and DLBCL patients (71.4%) were treated with chemotherapy. Of patients receiving chemotherapy, a higher proportion of mixed lymphoma and DLBCL patients received anthracycline-based combination chemotherapy regimens compared with MALT lymphoma (73% vs 71% vs 8%) whereas the proportion of mixed lymphoma and DLBCL patients was similar (p = 0.919). At a median follow-up of 37 months, the 5-year overall survival was 66.9%. The 5-year overall survival was 78% for MALT lymphoma, 84% for mixed lymphoma, and 45% for DLBCL. On univariate analysis, DLBCL histology, age, performance status, serum albumin, lactate dehydrogenase, bone marrow, number of extranodal sites, stage, and IPI score were prognostic for inferior survival. On multivariate analysis, DLBCL histology remained significantly prognostic for inferior survival, independent of chemotherapy regimen (hazard ratio (HR) 6.66, 95% confidence interval (CI) 2.01–21.41, p = 0.001). Mixed histology was not prognostic for inferior survival (HR 1.13, 95% CI 0.28–4.54, p = 0.868). Other factors prognostic for inferior survival were serum albumin <37 g/L (HR 3.22, 95% CI 1.11–13.22, p = 0.034) and treatment with non-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy (HR 4.89, 95% CI 1.67–14.36, p = 0.004). In conclusion, the clinical characteristics of mixed histology MALT lymphoma are similar to low-grade MALT lymphoma and significantly different from pure DLBCL. The prognosis of mixed histology MALT lymphoma is significantly better than pure DLBCL, independent of IPI and chemotherapy regimen, and pure DLBCL histology is independently prognostic of inferior survival outcome.