Gastrointestinal non-Hodgkin Lymphoma Research Articles

Characteristic endoscopic findings of gastrointestinal malignant lymphomas other than mucosa-associated lymphoid tissue lymphoma.

The gastrointestinal (GI) tract is the most common site of extra-nodal involvement for non-Hodgkin's lymphoma (NHL). The features of GI NHLs remain unclear. The aim of this study was to clarify endoscopic characteristics of GI NHLs. We retrospectively analyzed the morphological characteristics of 63 GI malignant lymphomas other than mucosa-associated lymphoid tissue lymphoma. Lesions were diagnosed between 2005 and 2020. Macroscopic findings were classified into five subtypes: superficial (S); protruding without ulcer (P); protruding with ulcer (PU); fungating (F); and multiple nodules (MN). Thirty-one lesions in the stomach were classified as S type in 3 cases (9.6%), P type in 6 (19%), PU type in 13 (42%), and F type in 9 (29%). In the stomach, the ulcerated phenotype was more frequent for diffuse large B-cell lymphoma (DLBCL) (89.5%) than for other histological types (41.7%; P = 0.01). In the intestine, 23 tumors were classified as S type in 4 cases (17%), P type in 1 (4%), PU type in 6 (26%), F type in 1 (4%), and MN in 11 (48%). Eleven of the 14 cases (78.6%) of intestinal follicular lymphoma lesions showed MN type. In the colon, eight tumors were classified as S type in 2 cases (25%), P type in 2 (25%), PU type in 1 (13%), and F type in 3 (38%). We have clarified the endoscopic features of GI NHL using macroscopic classifications. The ulcerated phenotype was the most frequent endoscopic finding for DLBCL.

Clinical Characteristics of 219 Patients with Primary Gastrointestinal Non-Hodgkin's Lymphoma

To analyze the clinical and pathological characteristics of primary gastrointestinal non-Hodgkin's lymphoma (PGI-NHL) patients, and to explore the factors affecting the patients' survival and prognosis. The clinical data of 219 patients with PGI-NHL diagnosed in our hospital from March 2009 to April 2016 was collected and retrospectively analyzed. Survival analysis was performed by using the Kaplan-Meier method. Log-rank test was used for comparison among the groups, and Cox regression was used for multivariate analysis. Among the 219 patients with PGI-NHL, 126 patients were males and 93 patients were females. 182 patients were IPI 0 to 2 and 37 patients were IPI 3 to 5. There were 205 cases (93.6%) of B cell phenotype and 14 cases (6.4%) of T cell phenotype. 140 patients (63.9%) were patients with primary gastric NHL, including 85 DLBCL and 19 MALT. 79 cases (36.1%) were patients with primary intestinal NHL, including 46 DLBCL, 4 MALT, 7 FL, 3 MCL and 4 Burkitt lymphoma. 23 cases were HP positive and received anti-HP therapy. 57 cases and 32 cases received surgery and chemotherapy respectively. 84 cases received combination treatment of surgery and chemotherapy and 11 cases received combination treatment of radiotherapy and chemotherapy. Overall survival (OS) of indolent B-cell non-Hodgkin's lymphoma was longer than that of invasive B-cell non-Hodgkin's lymphoma, which shows better prognose. Kaplan-Meier analysis showed that there was no difference between progression-free survival (PFS) and OS in the patients with different origin sites, age and sex. There was no significant difference in PFS between B-cell and T-cell-derived patients, whereas OS of B-cell-derived PGI-NHL patients was longer than that of T-cell-derived PGI-NHL patients. The OS and PFS of patients with IPI 0-2 were longer than those of patients with IPI 3-5. According to Lugano and Ann Arbor staging systems, there was no difference in prognosis of patients between phase I/II and III/IV. The prognosis of patients treated with surgery alone was worse than that of patients treated with combination therapy, and the prognosis of patients with surgery combined with chemotherapy was not significantly different from that of patients with chemotherapy alone. B-cell phenotype, indolent and low IPI score lymphoma indicate better prognosis, while that of different origin site, sex and age shows no different in prognosis. Surgery is used only for emergency case or pathological materials, and these patients should be treated with chemotherapy-based combined treatment.

Gastrointestinal (GI) Involvement with Non-Hodgkin's Lymphoma (NHL) Impacts Outcome; A Retrospective Single Tertiary Care Center Analysis of Clinico-Pathologic Characteristics Provides an Alternate Insight to Presentation and Outcome

Introduction Historically, the GI tract is involved in 10-15% of patients with newly diagnosed NHL. Despite being the predominant extranodal site for lymphoma involvement little is known about its pathophysiologic presentation given its overall rarity. Nonetheless its evaluation, diagnosis, prognosis and management is distinct from lymphomas at other sites hence their recognition irrespective of whether being the primary site is prudent. Methods We performed a single center retrospective study of patients to describe clinical characteristics and outcome of newly diagnosed NHL presenting with GI involvement defined by tissue biopsy between January 2003 and April 2019. Descriptive statistics were calculated by Fisher's exact test and Chi2 test for categorical variables and rank-sum analyses for continuous variables. Results We identified 91 patients with one patient presenting with 2 different GI NHL involvement. The median age at presentation was 70 years (range 33-92) and 61 were male. 41 (45%) patients had diffuse large B cell lymphoma (DLBCL) and 5 had concomitant low grade component, 28 (30.8%) mucosal associated lymphoid tissue (MALT) lymphoma, 10 (11%) mantle cell lymphoma (MCL), 4 (4.4%) enteropathy associated T cell lymphoma (EATL), 3 (3.3%) follicular lymphoma, 2 (2.2%) Burkitt's lymphoma and 2 (2.2%) EBV driven lymphoproliferative disorder (LPD). There were 1 case each of plasmablastic lymphoma and extranodal NK/T-cell lymphoma, nasal type. 37 with DLBCL and 2 with MCL had primary GI involvement. Gastric involvement was the commonest site, 43 (55.8%) cases followed by the large intestine, 22 (28.6%). PET-CT scan was done in 42 cases with high grade NHL; GI involvement by FDG avidity was noted in 23 cases and negative in 19 (45.2%). Serum albumin at presentation was available in all patient. Patients with presenting serum albumin < 25 g/L were more likely to die in the first 30-days post diagnosis; p<0.0001. A total of 20 patients died as a result of lymphoma; presenting serum albumin was significantly lower in that group (mean 29 g/L versus 37 g/L; p=0.0077). H pylori status was assessed by either antigen testing in stool and/or histology. This was known in 50 patients and was available by the 2 assessment methods in 31 cases but discordant in 17. A positive result was more likely noted with the antigen test. None of the T-cell LPD were H pylori positive. 12 of the 26 DLBCL and 9 (53%) of the 17 MALT lymphoma patients were H pylori status was known were positive. Nonetheless all H pylori negative MALT lymphoma cases were localized to the stomach and responded to triple eradication therapy and Rituximab monotherapy +/- surgical resection. In the DLBCL cohort, the cell of origin was known in 22 of 39 cases; 17 were non-germinal center phenotype. In the MCL cohort, all patients whom underwent surveillance endoscopies irrespective of symptoms and/or radiologic finding had histologic evidence of GI involvement with lymphoma with only 3 (18%) patients being symptomatic +/- had radiologic finding in keeping with GI involvement. Conclusions Despite the relatively small sample size this real-world data of GI lymphomas outlines some of its recognized features; advanced age at diagnosis, male prevalence and gastric involvement as the commonest site of involvement. Presenting serum albumin <25 g/L was associated with higher 30-day mortality highlighting its relevance and need for its inclusion as a biomarker in risk stratification of NHL where GI tract is involved. There is however several other significant findings not previously recognized; the incidence of GI involvement in MCL is much higher than reported; 100% in all patients whom have undergone endoscopy in our cohort suggesting that GI involvement is a feature of MCL. With only 53% of MALT lymphoma being H pylori positive which is much lower than previously described this renders the prototypical association of H pylori infection in MALT lymphoma pathogenesis void and suggests an alternate etiology. Nonetheless their response to antibiotic therapy with only minimal additional systemic therapy in some indicates that H pylori status does not necessary predict its efficacy and should be considered when treating MALT lymphomas irrespective of H pylori status. CT scans but also PET-CT scans are insensitive in detecting GI involvement even in high grade NHL. Endoscopy with random mapping biopsies even with normal macroscopic GI findings remains the gold standard. Disclosures No relevant conflicts of interest to declare.

Gastrointestinal non-Hodgkin lymphomas.

Although gastrointestinal (GI) tract is the most common extranodal site involved in non-Hodgkin lymphoma (NHL), primary gastrointestinal NHL (gNHL) is a rare problem which concerns about 10-15% of NHL patients and 30-40% of extranodal NHL patients. Lymphoid neoplasms may consist of mature B, T and (less commonly) extranodal NK/T cells. The most common diagnoses are diffuse large B-cell lymphoma and marginal zone lymphoma (MALT), but many other lymphomas may be found in the GI tract. There are a few well-known risk factors of gNHL and some of them affect treatment. The most frequent sites of occurrence are the stomach followed by small intestine and ileocecal region. In the last 2 decades, there has been a rapid development in the diagnosis, staging and management of GI lymphoma, but still some of such lymphomas, especially T-cell ones, are a therapeutic challenge. In this review, we present clinical and pathological features of GI lymphomas. We also describe the current status in diagnosis and treatment.

Analysis of Clinical Prognosis of 50 Cases of Primary Gastrointestinal Non-Hodgkin's Lymphoma

To analyze the clinical pathologic characteristics, diagnosis, treatment and outcome of patients with primary gastrointestinal non-Hodgkin's lymphoma(PGI-NHL). The clinical and pathological features of 50 cases of PGI-NHL were analyzed retrospectively, the Kaplan-Meier was applied to estimate the survival time of all the patients. The median age of patients was 58 years old, the cases of male patient were more than that of femal. The main clinical symptoms included pain and discomfort. The patients of Ⅰ-Ⅱstage accounted for 66%, DLBCL was most common. The clinical and pathological features were not significantly different between gastric and intestinal lymphoma. In 58% of the patients, surgery was the first choice for treatment. The median survival time was 74 months. The OS rates at 1-, 3- and 5-year were 78%, 65.9% and 61.8% respectively. Log-rank univariate analysis showed that age, sex, ECOG score, B symptoms, disease location and treatment methods all did not relate with OS, however, IPI, stage, LDH, cell phenotype and pathological type closely related with the OS. As lacking characteristic clinical manifestations of PGI-NHL, the DLBCL is the most common type. The prognosis of B cell lymphoma is significantly better than that of T cell lymphoma. The prognosis of PTCL is the worst.

Non–Hodgkin’s Lymphoma of the Appendix

AbstractInfiltration of the appendix as a manifestation of non-hodgkins lymphoma (NHL) at diagnosis or at relapse is a rare finding. We report the history of a patient with gastrointestinal non-hodgkin's lymphoma treated 8 years earlier who presented with symptoms suggestive of acute appendicitis secondary to relapsed NHL.Keywords: Non Hodgkins Lymphoma, Diffuse Large B Cell Lymphoma, appendix, PET CT

The Role of Surgery in the Clinical Management of Primary Gastrointestinal Non-Hodgkin's Lymphoma

Primary gastrointestinal non-Hodgkin's lymphoma (PGINHL) is a heterogeneous family of tumors, with treatment modalities including chemotherapy, surgery, and radiotherapy. Because the role of surgery in PGINHL remains disputed, this study aims to assess the impact of operative resection on survival. We used a pathology database to identify all cases of PGINHL diagnosed at a single academic-affiliated medical center from 1988 to 2013. Demographic and clinical data were abstracted from the medical record. We summarized the clinical courses of patients with PGINHL and then performed a survival analysis to compare overall and disease-free survival, stratified by demographic and clinical variables. We identified 33 patients diagnosed with PGINHL during the study period. Of 29 who subsequently received treatment at the institution, 15 initially underwent chemotherapy, 10 underwent surgical resection, and 4 underwent surgery for other reasons such as diagnosis without resection or management of disease complications. Three patients suffered surgical complications and two of these patients died. We found no difference in overall survival between patients receiving surgical resection and patients managed initially with chemotherapy. This case series supports a continued role for surgical resection in the management of patients with PGINHL, though anticipated benefits should be weighed against the risk of complications.

Abstract 2557: Immune modulating agent ibrutinib blocks T-helper 17 activation and release of IL-17A while preserving T-regulatory cell function

Abstract T-helper 17 (Th17) cells play a causal role in the pathogenesis of autoimmune and chronic inflammatory diseases. Chronic inflammation has been linked to various malignancies including gastrointestinal cancers, endometrial cancer, and non-Hodgkin lymphoma. Th17 cells secrete effector cytokines that are distinct from other divergent T-cell subsets. The namesake cytokine IL-17A is particularly pro-inflammatory and pro-fibrotic,driving the recruitment, activation, and migration of fibroblasts,endothelial cells, neutrophils, monocytes, and macrophages. Increases in IL-17A have been seen in patients with rheumatoid arthritis, Crohn's disease, multiple sclerosis, systemic lupus erythematosus, psoriasis, and encephalomyelitis. Dysregulated Th17 activity has also been linked to chronic graft versus host disease after allogeneic stem cell transplantation. Studies have shown that Th17 cells and T-regulatory (T-reg) cells are reciprocally regulated in the human body. T-regs produce anti-inflammatory cytokines that play an essential role in the prevention of autoimmune disease. At the molecular level Th17 cells rely upon ITK for T-cell receptor (TCR) induced activation. T-regs, however, utilize both ITK (IL2-inducible kinase) and RLK (resting lymphocyte kinase) establishing compensatory signaling cascades downstream of the TCR. Ibrutinib represents a first-in-class irreversible ITK inhibitor (Dubovsky et al: Blood July 25, 2013) with the established capacity to inhibit the activation of ITK dependent T-cells. Given our understanding of ITK and RLK expression in immune cell subsets we sought to determine whether ibrutinib could block Th17 cellular activation while preserving the functionality of T-regs. Our studies confirm that ibrutinib significantly blocks the TCR-induced activation of Th17 cells by up to 40% (p<0.0001). Moreover, ibrutinib pretreatment significantly limits the production of profibrotic IL-17A after ex-vivo TCR stimulation (p=0.030). Comprehensive and longitudinal murine in-vivo analysis revealed that up to four months of daily ibrutinib at 25mg/kg preserves T-reg numbers. Additional ex-vivo functional analysis demonstrated that the direct immunosuppressive capacity of highly purified(CD4+CD25hiCD127dimFoxP3+) human T-regs to limit activation and proliferation of healthy autologous CD4 or CD8 T-cells was not altered by ibrutinib. Our previously published and currently reported cumulative data along with an impressive clinical safety profile indicate that ibrutinib may therapeutically inhibit Th2 CD4 T-cell function and Th17 driven inflammation and autoimmunity while preserving the anti-inflammatory effects of T-reg cells, thereby justifying its investigation as an immune modulating therapy for cancer and graft versus host disease. Citation Format: Carrie S. Yang, Jason A. Dubovsky, Danielle L. Chappell, Bonnie K. Harrington, Samantha Jaglowski, Jennifer A. Woyach, Amy J. Johnson, Natarajan Muthusamy, John C. Byrd. Immune modulating agent ibrutinib blocks T-helper 17 activation and release of IL-17A while preserving T-regulatory cell function. [abstract]. In: Proceedings of the 105th Annual Meeting of the American Association for Cancer Research; 2014 Apr 5-9; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2014;74(19 Suppl):Abstract nr 2557. doi:10.1158/1538-7445.AM2014-2557

Clinicopathological analysis and prognostic factors of 11 patients with primary non-Hodgkin lymphoma of the small intestine in a single institute.

The gastrointestinal (GI) tract is the most common extranodal site of involvement in non-Hodgkin lymphoma (NHL). Primary GI NHL is frequently discussed in survival analyses. Primary intestinal NHL is significantly different from primary gastric NHL with regard to its clinical features, pathological subtype, treatment and prognosis. The small intestine is involved in lymphoma less often than the large intestine. The present study aimed to analyze the clinical and pathological characteristics of primary NHL of the small intestine and its prognostic factors. A retrospective analysis was performed on clinical data from 313 cases of NHL that occurred between 1995 and 2008 in the Tri-Service General Hospital (National Defense Medical Center, Taipei, Taiwan). Among these cases, 11 cases of primary NHL of the small intestine were identified. A Cox model was used to perform the multivariate analysis. The Kaplan-Meier method was used for the survival analysis. From the 11 patients with primary NHL of the small intestine, seven patients were male (63.6%) and four patients were female (36.3%). Furthermore, nine patients (81.8%) were diagnosed with B-cell lymphoma, of which five (45.5%) were also diagnosed with diffuse large B-cell lymphoma (DLBL). Abdominal pain and/or distention were present in six (54.5%) of the patients and jejunum involvement was also observed in six (54.5%) of the 11 patients. The mean overall survival (OS) time of the 11 patients was 27.2 months and the four-year survival rate was 36.3%. The mean OS time in the patients with jejunum involvement was shorter than in those without jejunum involvement (16.9 vs. 39.6 months), although this difference was not significant (P=0.657). Surgical treatment was performed on four of the six patients with jejunum involvement due to an acute abdomen or perforation-related peritonitis. The results of the present study indicate that DLBL is the most common subtype of primary lymphoma of the small intestine, and that the site involved in NHL may affect the potential for surgery in patients with intestinal lymphoma. Furthermore, patients with primary lymphoma of the small intestine have been found to have a poor outcome compared with those with lymphoma in other regions of the GI tract. In the present study, a similar trend was observed, however, the sizes of the subgroups of primary lymphoma of the small intestine were too small for individual analysis.

Successful Radiotherapy of a Rectal MALT B Lymphoma in an Adolescent

Purpose: We report the case of an adolescent with a rectal MALT lymphoma manifesting with hematochezia who was treated successfully with radiotherapy. Pediatric gastrointestinal (GI) malignancies are very rare. Of these, lymphomas are the most common. In adults, primary GI non-Hodgkin lymphomas (NHL) account for 4-20% of all NHL. WHO classifies NHL as Burkitt's, diffuse large B cell, mantle cell, follicular cell, and extranodal marginal zone mucosa-associated lymphoid tissue lymphoma (MALT). Colorectal lymphomas are very rare (10-20% of GI NHL and 0.2-0.6% of all colonic malignancies), and only 10-25% localize in the sigmoid and rectum. MALT lymphomas represent 5% of NHL and the majority occur in the stomach. MALT rectal lymphomas are extremely rare and have been treated with surgery, chemotherapy, antibiotics, or radiation therapy. A 16-year-old male presented with a history of painless hematochezia and constipation for 2 months. Past medical and family history were unremarkable. Rectal exam revealed redundancy of the posterior rectal mucosa and gross blood on the glove. The physical exam was otherwise normal. Colonoscopy was normal except for mild nodularity of the rectosigmoid, with cobblestoning of the first 5 cm of the rectum and the presence of two hypertrophic anal papillae. Biopsies of the first 5 cm of rectum, including the anorectal junction, showed an infiltrate of large atypical B cells CD20+, Pax 5+, CD 79a+, CD43+, MUM1+, BCL2+, BCL6+, CD5-, CD10-, BCL1-. Plasma cells were CD20- showing cytoplasmic IgA kappa restriction. CD79a was strongly expressed in plasmacytic cells and poorly in B cells. Ki67 proliferation index was low to moderate. EBER-ish was negative. The findings were consistent with marginal zone lymphoma with plasmacytic differentiation. PET scan showed a hypermetabolic uptake in the anal region (SUV 11.4). CT of the chest, abdomen and pelvis was negative. The patient received 24 GCE involved field proton beam radiotherapy in 12 fractions to the anorectal lesion. Pelvic and inguinal nodes were not irradiated. Repeat PET scan 5 months after radiotherapy showed decreased anal hypermetabolic uptake (SUV 4.4). Colonoscopy after 6 months showed mild nodularity of rectosigmoid. The anorectal margin had mild erythema and nodularity. Rectal biopsies showed benign lymphoid aggregates and the anorectal junction mucosa was normal. He reported occasionally minor anorectal bleeding when constipated. To our knowledge this is the first pediatric case of a rectal MALT lymphoma that was induced into remission by low dose proton beam radiotherapy. Although very rare, anorectal malignancies should be considered in the differential diagnosis of hematochezia in children and adolescents.

Primary Diffuse Large B Cell Lymphoma of the Rectum Mimicking an Arteriovascular Malformation: A Case Report and Review

Introduction: Lymphomas are malignant neoplasms of the lymphocyte cell lines. They mainly involve lymph nodes, spleen, and other non-hemopoietic tissues, and are classified as either Hodgkin's or non-Hodgkin's lymphomas (NHL) of B-lymphocyte or T-lymphocyte origin. According to estimates, in 2008, 66,120 new cases of NHL occurred in the United States. Gastrointestinal NHL account for 4-20% of all NHL. Approximately 0.2-0.6% of malignant lymphomas arise in the colorectal tract. Primary rectal NHL is rare, accounting for 0.2% of all rectal malignancies. We present a patient with a history of multiple admissions with presumed rectal arteriovascular malformations (AVMs) who was ultimately diagnosed with primary diffuse large B cell lymphoma of the rectum (DLBCL). Case: An 84-year-old Chinese male with past medical history of Hepatitis B and BPH presented to the ER with recurrent episodes of hematochezia. He denied abdominal pain, nausea, or vomiting. Previous colonoscopy for evaluation of the same symptoms had revealed only internal hemorrhoids and AVMs. He subsequently underwent a repeat colonoscopy which revealed a 3-cm ulcerated lesion immediately above the anal verge with persistent, active bleeding from the distal margin of the mass. APC thermal therapy was applied with adequate hemostasis. Biopsy and immunochemistry report was positive for CD5, CD20, PAX-5, BCL-2, BCL-6, consistent with DLBCL of the rectum. He subsequently underwent combined R-CHOP and radiation therapy with good results. Discussion: A thorough search of Pubmed and MEDLINE revealed fewer than 75 reported cases of primary B cell lymphoma of the rectum. The lack of specific complaints makes the diagnosis difficult to establish. Typically, the mean age at diagnosis is 55 years. Men are affected twice as often as women. The most common symptoms in more than half of patients are abdominal pain, weight loss, or changes in bowel habits. Lower gastrointestinal bleeding can be found in 13-20% of patients. The CHOP chemotherapeutic regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) remains the first line therapy, with surgery or radiation in some cases. Recent studies suggest that the addition of rituximab may lead to improved overall survival as well as disease progression. Conclusion: We present the case of an 84-year-old male with a history of multiple admissions for lower GI bleeding secondary to presumed AVMs, with a subsequent diagnosis of DLBCL. This rare malignancy can grow rapidly and should be included in the differential for recurrent lower gastrointestinal bleeding.

P-0128 HIV-Associated Lymphoma of the Gastrointestinal Tract in the Combination Antiretroviral Therapy Era

ABSTRACT Introduction HIV-associated non-Hodgkin's lymphoma (NHL) remains the most common HIV-associated malignancy despite the advent of combination antiretroviral therapy (cART). The gastrointestinal (GI) tract is one of the most common extranodal sites. GI NHL can be divided into primary GI lymphoma and secondary involvement from diffuse nodal disease. The median survival time of HIV-associated lymphoma with GI tract involvement in the pre-cART era was 4–10 months. The aim of this study is to investigate its characteristics and survival outcomes in the cART era. Methods We retrospectively analyzed the medical records of patients (including 2023 HIV-infected patients) diagnosed with GI lymphoma between April 2000 and December 2011 at Osaka National Hospital. Results Seventy-four patients had been diagnosed with GI lymphoma (primary or systemic lymphoma with GI involvement) with (14 patients) or without (60 patients) HIV infection. Of the 2023 HIV-infected patients, 14 had been diagnosed with GI lymphoma. One of these patients had been diagnosed with primary gastric diffuse large B-cell lymphoma (DLBCL) on autopsy. Of the 13 patients, 5 had primary GI lymphoma and 8 had systemic lymphoma with GI tract involvement. The median age at presentation was 44 years (range, 32–72 years), median CD4 lymphocyte count was 101/mm3 (range, 4–560/mm3), and median HIV RNA viral load was 52000 copies (range, Conclusion In HIV-associated lymphoma with GI tract involvement, survival is better in the cART era than the pre-cART era. The overall survival of HIV-infected patients with GI aggressive B-cell lymphomas was similar to that of HIV-negative patients with such lymphomas.

Clinicopathologic features of primary gastrointestinal non-Hodgkin's lymphoma

The clinical data of 26 cases with pathologically confirmed primary gastrointestinal nonHodgkin's lymphoma (PGINHL) admitted to our hospital from January 2001 to December 2010 were retrospectively analyzed.Among 26 patients,12 cases occurred in stomach,9 cases in small intestine,4 cases in colon and 1 case in multiple sites.The most common symptoms were abdominal pain (73％),followed by gastrointestinal bleeding (23％) and abdominal mass (15％).The main pathological types were MALT lymphoma (35％),diffuse large B-cell lymphoma (35％),and enteropathy-type T-cell lymphoma ( 15％ ).There were 14 patients in stage Ⅰ,6 in stage Ⅱ,1 in stage Ⅲ and 5 in stage ⅣV.Radical surgery and/or chemotherapy was the main therapy for PGINHL. Key words: Lymphoma; Non-Hodgkin; Retrospective study

Increasing Incidence Rates, Distribution and Histological Characteristics of Primary Gastrointestinal Non-Hodgkin Lymphoma in a North American Population

The incidence of primary extranodal non-Hodgkin lymphoma (NHL) of the gastrointestinal (GI) tract has been on the rise. To determine the incidence of primary GI NHL and distribution according to site and histological type in a large North American adult population over a 10-year period. All diagnoses of GI NHL made between January 1999 and January 2009 were reviewed using a regional pathology database. Patients ≥18 years of age living within health region boundaries were included. Age- and sex-adjusted incidence rates of GI NHL according to GI site and histological type over a 10-year period were calculated and compared. A total of 149 cases of primary GI NHL were identified during the study period. Age- and sex-adjusted yearly incidence rates ranged from 0.13 per 100,000 in 1999, to 2.39 per 100,000 in 2007. Histological distribution (47% diffuse large B cell lymphoma, 24% extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type, 8% follicular and 5% mantle cell) and site distribution (47% stomach, 26% small bowel, 17% colon) were obtained with increasing annualized incidence rates for each of these sites over time. Remaining cases included multiple GI sites of involvement (9%) and esophagus (0.7%). Population-based GI NHL incidence rates in the present study were higher than those described elsewhere in North America and Europe. Nearly one-half showed high-grade (diffuse large B cell lymphoma) histology at diagnosis. Incidence rates for the colon exceed those described in other studies worldwide. Because the majority of GI NHL are diagnosed on endoscopic biopsy, clinicians and pathologists must be vigilant of this entity.

Jejunal Stricture

The use of intensive chemotherapy has led to remarkable improvements in the treatment of high-grade B-cell Non-Hodgkin lymphoma (NHL); however, it is associated with significant side effects such as myelosuppression and mucositis. Gastrointestinal NHL rarely leads to the development of aneurysmal dilatation of the bowel, as desmoplastic reaction is not a feature of NHL. Strictures and fibrosis are not a manifestation of NHL involvement. Here, we report a child with primary gastrointestinal B-cell NHL who presented with jejunal stricture developing as a sequela of severe chemotherapy-induced mucositis. The patient improved with surgical resection of stricture and end-to-end anastomosis.

Clinical prognostic analysis of 116 patients with primary intestinal non-Hodgkin lymphoma

The gastrointestinal tract is the most common extranodal invasion site of non-Hodgkin lymphoma (NHL). Primary gastrointestinal NHL is often discussed together in most survival analyses. Primary intestinal NHL is significantly different from primary gastric NHL with regard to clinical features, pathological subtype, treatment, and prognosis. In this article, we analyzed clinical and pathological characteristics of primary intestinal NHL, and we also explored prognostic factors for primary intestinal NHL. A retrospective analysis was carried out on clinical data from 116 cases of confirmed primary intestinal NHL. The Kaplan-Meier method was used for the survival analysis. A Cox model was used for a multivariate analysis. In 116 patients with primary intestinal NHL, 79 patients were men (68.1%) and 37 patients were women (31.9%). In the cases used in this study, 68 were B-cell NHL and 48 were T-cell NHL. The age, incidence of intestinal obstruction, B symptom and performance status (PS) were closely related with pathological subtype. One-year and two-year survival rates were 76.7 and 58.3%, respectively. The log-rank univariate analysis showed male patients, PS score greater than or equal to two, hypoproteinemia, intestinal perforation, T-cell type, late stage (III/IV), no radical surgery, and no chemotherapy had relatively poor prognoses. Cox multivariate analysis shown that gender (95.0% CI 0.218-0.721), pathological subtype (95.0% CI 1.484-4.179), and radical surgery (95.0% CI 0.110-0.394) were independent prognostic risk factor for primary intestinal NHL. Male patients, T-cell intestinal lymphoma, and no radical surgery had rapid clinical processes and poor prognoses.

Primary gastrointestinal non-Hodgkin’s lymphoma: clinicopathological and prognostic analysis

The objective is to investigate the association between pathological type and clinical features, response to treatment and prognosis of primary gastrointestinal Non-Hodgkin's lymphoma (PGINHL). The clinicopathologic features, response to treatment and survival of 124 patients with PGINHL, were investigated retrospectively. Ninety-one patients were treated with surgery, most of which included combined therapy, while 32 patients received chemotherapy alone. Survival analysis was performed by Kaplan-Meier and Cox regression method. The most common immunophenotype was B-cell subtype. In 115 (92.7%) patients of B-cell lymphoma, mucosa-associated lymphoid tissue lymphoma (MALToma) and diffuse large B-cell lymphoma (DLBCL) were 55 and 50 patients, respectively. The patients of two pathological types had different clinical features including stage, B symptoms, sites of tumor, distant involvement, International Prognosis Index Score, size of tumor, and response to treatment. Both overall survival curve and multiple Cox regression analysis indicated that pathological type was statistically significant. The pathological subtype of PGINHL was an important prognostic factor. The patients with MALToma appear to have more favorable prognosis than those with DLBCL.

Long term follow-up of primary B and T cell non-Hodgkin's lymphoma (NHL) of the gastrointestinal (GI) tract

e19516 Background: Anthracycline based chemotherapy is the treatment of choice for aggressive primary lymphomas of the GI tract, with surgery reserved for management of complications. We report long term follow up of 71 cases of primary GI NHL treated with chemotherapy and/or surgery. Methods: Cases were identified from the institutional histology database & records reviewed. Results: 71 patients over an 24 year period were identified; 43 (61%) male, 28 (39%) female. Median age at diagnosis was 60 (15–83). 52 (73%) were DLBCL, 11 (16%) were T-cell, 8 (11%) were MALT. The 8 patients with MALT were treated with single agent chemotherapy; 7 (88%) are alive at median follow up of 8.5 years (2–16). Of the aggressive lymphomas (63), all patients with T cell lymphoma had small bowel as primary site and histological evidence of celiac associated enteropathy, even in the absence of known celiac disease. Primary sites of DLBCL were stomach 35 (67%), small bowel 11 (21%) & colon 6 (12%). 39 (62%) patients underwent surgery at diagnosis due to acute presentation with perforation, bleeding or obstruction, or to obtain histology. Following confirmed diagnosis, 61 patients received anthracycline based chemotherapy. 2 patients with T cell lymphoma presented with perforation, were treated with surgery only and died of rapid disease progression. Of the 63 patients with aggressive NHL, 37 (59%) remain alive & disease free at median follow up of 13 years (1–24). 35 (67%) patients with DLBCL are alive & disease free. Only 2 (18%) of the T cell lymphomas are alive & disease free. 5 deaths in the DLBCL group were not related to cancer or treatment. All deaths in the T cell group were due to progressive disease. There was no difference in survival between patients treated with chemotherapy only and those who also underwent surgery. Conclusions: Patients with aggressive primary B cell GI NHL have almost 70 % survival following anthracycline based chemotherapy. However, in contrast, coeliac enteropathy associated T-cell lymphomas present with rapidly progressive disease & have a survival of < 20% with chemotherapy and/or surgery. A novel therapeutic approach is required to improve outcome in this group. No significant financial relationships to disclose.

Changing Epidemiology and Risk Factors for Gastrointestinal Non-Hodgkin's Lymphoma in a North American Population: Population-Based Study

To assess the incidence, risk factors, and endoscopic presentation of gastrointestinal non-Hodgkin's lymphoma (GI NHL) in a large predominantly urban adult population sample. A comprehensive database review of all diagnoses of GI NHL in the Calgary Health Region over a 5-yr period (1999-2003) was undertaken. Longer-term data from a population-based HIV database (1985-2004) were also reviewed. A regional pathology database was used to corroborate case identification. All patients 18 yr of age or older were included. Age- and gender-adjusted incidence rates were calculated. Within the HIV-positive population, incidence rates were compared over time. Endoscopic appearances were assessed and compared. Fifty-six GI NHL cases occurred during the study period. The age- and gender-adjusted annual incidence of GI NHL was 1.73 per 100,000 in the study population. The majority were diffuse large B-cell histology (54%), followed by lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) (29%). Increasing age, history of kidney transplant, and H. pylori positivity in MALT lymphoma were identified as risk factors. Within the HIV-positive population, a highly significant drop in GI NHL was seen over time, with an incidence of 3.86 per 1,000 patient-years in 1985-1989 compared to zero cases in 2000-2004, despite a greater prevalence of HIV disease (P < 0.0001 for trend). MALT lymphoma was less likely to manifest as a mass on endoscopy versus other presentations (P < 0.05). Population-based GI NHL incidence rates in Calgary are higher than those described elsewhere in North America or in Britain. The incidence of GI NHL within the HIV population has virtually disappeared, presumably due to the advent of highly active retroviral therapy.

B-Zell-Non-Hogdkin-Lymphom des Duodenums

Gastrointestinale Non-Hodgkin-Lymphome (NHL) sind seltene Neoplasien des Abdomens. Wir berichten über einen Patienten, bei dem durch eine Gastroskopie ein follikuläres Lymphom im Duodenum gefunden wurde. Die Ausbreitungsdiagnostik erbrachte einen weiteren Lymphombefall im Bereich der Tonsillen. Damit handelt es sich um ein B-Zell-NHL im Stadium IV.