Endoscopic features and prognoses of mantle cell lymphoma with gastrointestinal involvement

AIM: To evaluate the endoscopic manifestations and prognoses of gastrointestinal (GI) mantle cell lymphoma (MCL). METHODS: A database search at the Department of Pathology of Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences revealed 57 MCL patients with GI involvement. Clinical records were available for 35 of the 57 patients from 21 institutions, and those 35 patients were enrolled in this study. We summarized the gross types of endoscopic features, event-free survival...     

Non-multiple lymphomatous polyposis form of mantle cell lymphoma in the gastrointestinal tract

Mantle cell lymphoma (MCL) comprises 2.5%–7% of all non-Hodgkins lymphomas, and the gastrointestinal tract is involved in about 20% of cases. Multiple lymphomatous polyposis (MLP) is an uncommon disease that is regarded as the intestinal form of MCL. We present a rare case of gastrointestinal MCL without MLP, and demonstrate that rituximab was effective for the treatment of this patient. A 61-year-old man presented with continuous diarrhea and hematochezia for a period of 5 months. Superficial lymph nodes were not palpable, but both tonsilla were enlarged. The level of soluble interleukin (IL)2-receptor was 3480U/ml (normal <500U/ml). Colonoscopy showed diffuse redness with erosion, without observation of any venous capillary, with these findings continuing from the rectum to the ileum. Upper gastrointestinal endoscopy showed a slightly rough gastric mucosal surface, and chicken-skin like mucosa was observed in the second portion of the duodenum. Small-to-medium size lymphoma cells were seen histologically from the tonsilla to the rectum. The lymphoma cells were immunohistochemically positive for CD5, CD20, CD79a, and cyclin D1. Polymerase chain reaction analysis revealed a chromosomal translocation t(11;14)(q13;q32) in the bcl-1 gene. We diagnosed this as a case of MCL from these findings. For treatment, the patient received a total of ten courses of combination chemotherapy consisting of cyclophosphamide (1000mg), doxorubicin (70mg), vincristine (2mg) and prednisolone (50mg) (CHOP), which led to a partial remission. However, 2.5 years later, massive infiltrations of the lymphoma cells were found in the colon and stomach. As the infiltrating lymphoma cells expressed CD20 molecules on their surfaces, the patient was treated with a chimeric anti-CD20 monoclohal antibody, rituximab, which showed significant efficacy, and a second partial remission was achieved.     

Endoscopic findings in upper gastrointestinal tract in patients with liver cirrhosis

151 patients suffering from the cirrhosis of the liver underwent a prospective endoscopic examination of the upper digestive tract. The most frequent diagnoses in the group with the cirrhosis of the liver included oesophagus varices (64.9%), portal hypertension gastropathy (45.7%) and the peptic ulcer of the gastroduodenum (25.8%). A normal diagnosis in the endoscopy of the upper digestive tract was found only in 8.6%. Other diagnoses comprised reflux oesophagitis (13.2%), diaphragm hiatus hernia (12.6 %), duodenogastric reflux (8.6 %), gastric antrum erosion (4.6 %), aphthic gastropathy (3.3 %), rhagades of the cardium (2%), gastric polyp (1.3%), mycotic oesophagitis, gastric carcinoma, oesophagus carcinoma and oesophagus achalasy (0.7% each). Further on the study discusses possible causes of the high incidence of peptic ulcers in the patients with the cirrhosis of the liver. All findings are correlated with literary data.     

Central nervous system involvement in patients with mantle cell lymphoma

 In small cell lymphomas, central nervous system (CNS) involvement has been considered to be very rare. Mantle cell lymphoma (MCL) is a distinct subtype of non-Hodgkin's lymphomas consisting of small or intermediate lymphatic B-cells. It has a poorer prognosis than the other small cell lymphomas. Only a few MCL patients with CNS involvement have been reported in the literature to date. We analyzed retrospectively the incidence, clinical characteristics, and outcome of CNS involvement in 94 patients with confirmed MCL treated at one center from 1980 to 1997. Four of the 94 patients (4%) developed CNS lymphoma during the median follow-up of 51 months. The diagnosis was based on clinical, cytological and radiological findings. CNS involvement appeared at 4.6, 56, 66, or 86 months from the diagnosis of MCL. All patients had neurological symptoms and a leukemic disease; two cases were seen with a blastoid morphology. Malignant lymphatic cells were detected in spinal fluid in all cases and parenchymal infiltrations in brain in two. All patients were treated with intrathecal chemotherapy, without response. Survival time after diagnosis of CNS lymphoma ranged from 18 to 55 days. At diagnosis, no adverse prognostic factors predictive of CNS lymphoma were found. CNS involvement was associated with a progressive leukemic disease as a late event or a blastoid transformation. The prognosis of MCL patients with CNS involvement is poor. Received: July 30, 1998 / Accepted: November 12, 1998     

Endoscopic findings of gastrointestinal involvement in Chinese patients with Behcet’s disease

AIM:To report the incidence,clinical features and outcomes of gastrointestinal(GI)involvement in Behcet’s disease(BD).METHODS:A total of 168 consecutive patients with BD were screened and upper and lower GI endoscopies were performed in 148 patients.Four hundred age-and sex-matched controls were enrolled for comparison.RESULTS:Fifty-two(35.1%)patients had GI lesions.After a mean follow-up of 10 mo,ileocecal ulcers had been confirmed in 20 patients,including active ulcer(s)in 18 patients,but no ileocecal ulceration was found in controls.GI symptoms were present in 14 patients with active ulcer(s),while 4 patients with smaller ulcer were asymptomatic.Endoscopic features of ileocecalulcer were:a single ulcer(50%),larger than 1 cm in diameter(72.2%),and round/oval or volcano-type in shape(83.3%).Compared with patients without GI involvement,less ocular lesions,lower levels of albumin,erythrocyte count and hemoglobin,and higher levels of C-reactive protein and erythrocyte sedimentation rate were confirmed in the intestinal BD group.Four patients had esophageal ulcers in the BD group but no case in controls.The other endoscopic findings were similar between the two groups.The prevalence of Helicobacter pylori infection was similar in both groups.Most patients received an immunomodulator and responded well.CONCLUSION:GI lesions commonly occur in Chinese BD patients.The most frequently involved area is the ileocecal region.Esophageal ulcer might be a rare but unique lesion.     

A rare case of mantle cell lymphoma as lymphomatous polyposis with widespread involvement of the digestive tract

Lymphomatous polyposis of the gastrointestinal tract is rare. It refers to a heterogeneous group of small B-cell lymphomas including mantle cell lymphoma, follicular lymphoma and MALT lymphoma. It is characterized by the presence of multiple lymphomatous polyps along one or more segments of the digestive tract. Clinical symptoms are non-specific. We herein report the case of a 74-year old man initially admitted for an upper and lower gastrointestinal endoscopy to explore a positive Hemoccult test. The endoscopy revealed multiple polyps all along the gastrointestinal tract. Histopathological study showed a diffuse lymphomatous proliferation of small B-cells whose immunohistochemical features were compatible with a mantle cell lymphoma. Tumoral B-cells showed a positivity of cyclin D1 markers but negativity for CD5. Immunochemotherapy with R-CHOP (rituximab, cyclophosphamide, adriamycine, vincristine and prednisone) was initiated. Based on this case study, the pitfalls of gastrointestinal tract lymphomatous polyposis diagnosis, prognosis and treatment options are discussed.     

Bone marrow and peripheral blood involvement in mantle cell lymphoma

The peripheral blood smears, bone marrow aspirates and biopsies of 46 patients with mantle cell lymphoma were reviewed. The diagnosis of mantle cell lymphoma was established in all cases on extramedullary tissue samples using standard morphologic, phenotypic and molecular genetic criteria. 27/35 patients (77%) had circulating lymphoma cells (median 20% of all circulating white blood cells; range 5–90%) identified by morphology at some point during the course of their disease. No statistical difference in survival was detected in patients with or without peripheral blood involvement. Lymphoma was identified in bone marrow aspirate specimens from 33/40 patients (83%) and in bone marrow biopsy specimens from 39/43 patients (91%). The pattern of marrow biopsy involvement was nodular (31 cases; 82%), interstitial (19 cases; 50%), paratrabecular (17 cases, 45%) and diffuse (12 cases; 32%). Although the median survival of patients with ≥ 50% bone marrow involvement was 13 months, and the median survival of patients with ≤ 50% was 49 months, no statistically significant differences between these small subgroups were observed. Mantle cell lymphoma frequently involves the peripheral blood and bone marrow. Its appearance is distinctive but variable, and immunophenotypic studies as well as morphologic confirmation by a biopsy of tissue other than bone marrow is still required for diagnosis.     

Endoscopic polypectomies in upper gastrointestinal tract

Nine polyps retrieved from the upper gastrointestinal tract by endoscopic polypectomy in nine patients are reported. Polyps were removed from the distal esophagus (1), stomach (7) and duodenum (1). Dysphagia, obstructive jaundice and upper gastrointestinal bleeding were the presenting features in four patients. In five patients gastric polyps were detected incidentally at endoscopy. Of the nine polyps, six were adenomas, two were hyperplastic polyps and one in the esophagus was inflammatory. All the polyps could be retrieved completely and there were no complications. Thus polyps do occur in the upper gastrointestinal tract in India; their electrosurgical removal is easy and safe and allows histopathological examination of the entire polyp.     

Endoscopic findings in the upper gastrointestinal tract of faecal occult blood-positive, colonoscopy-negative patients

BACKGROUND: Detection of faecal occult blood is recommended for colorectal cancer screening in average risk populations. However, many subjects do not have any cause found in the colon to account for the occult blood loss. AIMS: To determine the prevalence of upper gastrointestinal tract disease in faecal occult blood-positive, colonoscopy-negative patients. PATIENTS AND METHODS: Retrospective audit of 99 patients (56 females; mean age 60 years, range 18-83) who underwent same-day colonoscopy and upper gastrointestinal endoscopy over a 2-year period. RESULTS: Fifty-two of the 99 patients had a normal colonoscopy, 16 had diverticulosis and 2 had hyperplastic polyps; these 70 patients comprised the colonoscopy-negative group. Significant upper gastrointestinal tract disease was noted in 25 (36%) of the colonoscopy-negative group compared with 10 (34%) of the 29 colonoscopy-positive group (p=ns). Most of the upper gastrointestinal tract lesions identified were benign. Within the colonoscopy-negative group, patients with anaemia or upper gastrointestinal tract symptoms had a higher prevalence of positive findings in the upper gastrointestinal tract, but this association was not statistically significant. CONCLUSIONS: Endoscopic examination of the upper gastrointestinal tract in faecal occult blood-positive individuals reveals mostly benign disease, with an equal prevalence in colonoscopy-negative and colonoscopy-positive patients. Routine performance of upper gastrointestinal endoscopy in faecal occult blood-positive individuals is not indicated and should be undertaken only for appropriate symptoms.