Isolated jejunal Crohn's disease in a young adult presenting as fever of unknown origin

A 27-yr-old man was referred for fever, weight loss, fatigue, and occasional mild epimesogastric pain without diarrhea or vomiting. Laboratory tests were suggestive of an active inflammatory disease but serological, bacteriological, viral searches, markers of autoimmunity, and neoplasia were all negative. The following were also negative: ultrasonography; conventional x-rays; CT scans; esophagogastroduodenoscopy, pancolonoscopy with ileoscopy; cytohistology including duodenum and ileocolon. Empiric antibiotic regimens failed to control the temperature. Small bowel enema disclosed multiple proximal jejunal strictures. Jejunoscopy revealed erythema, friability, linear ulcerations, stenosis, and dilation in the proximal jejunum. Multiple directed biopsies showed inflammatory changes devoid of any specific features. The patient received steroid treatment and his temperature normalized. Six months later, he was readmitted on account of intestinal subocclusion that was managed conservatively. A few days later urgent laparotomy was performed with peritoneal lavage, repair of double perforated proximal jejunal ulcers, and stricturoplasty. Surgical jejunal biopsy confirmed the results of enteroscopic biopsies. The patient is presently without fever, in the absence of steroid treatment.
There have been no reports of cryptogenic fever due to isolated jejunal Crohn's disease in the recent literature. Our patient's clinical picture resembled disease as seen in older children and adolescents, in whom it is a difficult diagnosis owing to the absence of diarrhea. In adults with Crohn's disease isolated jejunal involvement represents approximately 1% of cases.
A thorough small bowel investigation is warranted in young adults with cryptogenic fever and low serum protein levels, even in the absence of major gastrointestinal complaints.     

Refractory sprue syndrome with clonal intraepithelial lymphocytes evolving into overt enteropathy-type intestinal T-cell lymphoma

INTRODUCTION: Recently, patients with refractory sprue have been shown to contain a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes in their intestine. Whether this signifies early enteropathy-type intestinal T-cell lymphoma (EITCL) or a reactive condition is not clear. We report on a patient presenting with the findings of refractory sprue who subsequently developed overt EITCL. MATERIAL AND METHODS: Duodenal biopsies from 1997 (refractory sprue) and duodenal and jejunal biopsies from 1998 (intestinal T-cell lymphoma) were compared by immunohistochemistry and PCR for the detection of T-cell receptor (TCR)-gamma gene rearrangements. Clonal PCR products were sequenced. RESULTS: The duodenal biopsies from both 1997 and 1998 and the jejunal tumor biopsy showed villus atrophy and an increase of intraepithelial lymphocytes with an abnormal immunophenotype (CD3+, CD4-, CD8- and TCR-beta-). In all duodenal specimens including the one from 1997, and the jenunal tumor biopsy, an identical clonal amplificate was detected by enzymatic amplification of the TCR-gamma gene. CONCLUSION: These data suggest that refractory sprue containing a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes may represent an early manifestation of EITCL. The detection of immunohistochemical negativity for several antigens normally found on intraepithelial lymphocytes such as CD8 or the TCR-beta chain in combination with clonal T-cell populations by PCR may be helpful in identifying refractory sprue with a malignant transformation.     

Intestinal T-cell lymphoma associated with celiac disease masked by cavernous lymphangioma.

We present the case of a patient admitted to our emergency ward with a clinical setting of acute abdominal pain and a history of cavernous lymphangioma, diagnosed in another center by exploratory lapartomy. The patient presented complete analysis including serology tests, as well as an abdominal CT scan that revealed multiple large size retroperitoneal cysts. In view of the clinical symptomatology and results of the tests, a second CT scan was carried out upon admission. As a result of the findings obtained, a second exploratory laparotomy was carried out in which intestinal resection of the perforated jejunal loop and largest cysts was performed.Pathological anatomy diagnosed an intestinal lymphoma associated with enteropathy and abdominal cysts compatible with cavernous lymphangioma. In this work we describe both pathologies, the most characteristic aspects are analyzed and the etiology and possible relation between both entities is discussed.     

Free perforation of the small intestine in collagenous sprue

A 67-year-old man with celiac disease developed recurrent diarrhea, profound weakness and weight loss, with evidence of marked protein depletion. His clinical course was refractory to a strict gluten-free diet and steroid therapy. Postmortem studies led to definition of unrecognized collagenous sprue that caused ulceration and small intestinal perforation. Although PCR showed identical monoclonal T-cell populations in antemortem duodenal biopsies and postmortem jejunum, careful pathological evaluation demonstrated no frank lymphoma. Rarely, overt or even cryptic T-cell lymphoma may complicate collagenous sprue, however, small intestinal ulcers and perforation may also develop independently. The dramatic findings here may reflect an underlying or early molecular event in the eventual clinical appearance of overt T-cell lymphoma.     

Chronic enterocyte infection with coronavirus

A man with a gastrojejunostomy and intestinal malabsorption was found to be excreting large numbers of coronavirus-like particles in his stools over a period of at least eight months. Coronavirus-like particles were found in vesicles in degenerating jejunal enterocytes in all of five jejunal biopsies. In a review of electron micrographs, similar structures were found in biopsies from three of 12 patients with classical chronic tropical sprue and in one patient with a sprue-like syndrome associated with agammaglobulinaemia. The hypothesis is advanced that infection with this virus may produce enterocyte damage and may be one cause of the syndrome of tropical sprue.     

Gastric infiltration of diffuse large B-cell lymphoma： Endoscopic diagnosis and improvement of lesions after chemotherapy

Diffuse large B-cell lymphoma （DLBCL） is the most common histologic subtype of the non-Hodgkin’s lymphoma （NHL） accounting for about 40% of all NHLs. This is a case report about the endoscopic appearance of a DLBCL with infiltration to the stomach in a 39-year-old female. She had a 6-mo history of lumbar and left upper quadrant pain with intermittent episodes of melena. A computer tomograghy （CT） scan showed mural thickening of the gastric antrum. Endoscopic examination revealed multiple gastric ulcers. Definite diagnosis could be made by endoscopic biopsies and the patient had a good response to chemotherapy. This response correlated well with a further endoscopic follow-up. A follow-up endoscopic examination could be considered to evaluate a good response to chemotherapy in DLBCL patients with secondary gastric dissemination.     

Jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception

A 73-year-old male was admitted to our institution with complaints of nausea, vomiting, and abdominal distension. Plain abdominal computed tomography (CT) suggested intussusception in the jejunum. Enhanced abdominal CT revealed the ‘target-like’ sign and ultrasonography revealed the ‘multiple concentric ring’ sign; therefore, a diagnosis of entero-enteric intussusception was made. The small intestinal obstruction and cause of the intussusception were not evident. The patient was treated conservatively with fasting and transfusion therapy to prevent intestinal obstruction. However, with no spontaneous resolution of intussusception, surgical treatment was decided. The operative findings revealed a jejunal tumor about 30 cm from the Treiz ligament, and the jejunum including the tumor with a 5 cm margin were partially resected. The resected tumor was a 35 × 50 mm soft mass spreading laterally with nodules. The pathological examinations revealed tubulovillous adenocarcinoma in the adenoma. Intussusception is rare in adults compared to children. About 45 % of cases of intussusception in adults are due to small intestinal tumors such as malignant lymphoma or lipoma, but a tubulovillous adenocarcinoma with adenoma is a rare cause of intussusception. We present a rare case of jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception.     

Intestinal NK/T cell lymphoma: A case report

BACKGROUND The incidence of intestinal NK/T cell lymphoma(NKTCL) is extremely low, and the clinical symptoms are atypical, which makes it difficult to distinguish this disorder from Crohn's disease(CD), T lymphocyte proliferative disease, and other immune disorders. The misdiagnosis rate is high, and the patient's prognosis is poor.CASE SUMMARY In this case, the patient had repeated high fever, colonoscopy revealed multiple ulcers, and the initial diagnosis was CD. The patient's condition did not improve after treatment with hormones and infliximab, and she eventually died. Positron emission tomographic-computed tomographic and B-ultrasound were performed in our hospital and showed that multiple lymph nodes were enlarged. Immunohistochemi-stry showed that CD3 and Epstein-Barr virus encoded RNA expression was positive. Colonoscopy, tissue biopsy, and histopathology showed intestinal focal mucosal infiltration of heterotypic lymphocytes with an abnormal immune phenotype. On the basis of the patient's medical history, auxiliary examination, and pathological findings, digestive physicians and pathologists gave the diagnosis of NKTCL.CONCLUSION Clinicians need to improve their comprehensive knowledge of NKTCL, and combination of clinical symptoms, histological characteristics, as well as colonoscopy biopsies should be considered to improve the diagnosis and thereby reduce misdiagnosis.     

Endoscopic diagnosis of lower intestinal lesions of leukaemia and malignant lymphoma

There have been only a few endoscopic studies with respect to lower intestinal lesions of leukaemia and malignant lymphoma, although there have been many autopsy studies of these lesions. The aim of this study was to clarify these lesions using endoscopy. Colonoscopy was performed on 11 of 341 patients with leukaemia and on 32 of 105 patients with malignant lymphoma for frequent diarrhoea, anal bleeding or abnormal findings on barium enema examination, between April 1984 and September 1994. In eight of the 11 patients with leukaemia on whom endoscopy was performed, nine lesions were found; aphthoid ulcers, small ulcers or large tumours due to leukaemic infiltration were found in five, and colorectal adenoma was found in only one patient. Antibiotic-associated haemorrhagic colitis or pseudomembranous colitis was found in one patient each. In 10 of the 32 patients with malignant lymphoma, 11 lesions were found. The following were found in one patient each: large lymphomatous tumours, a large lymphomatous ulcer, multiple small polypoid lesions, multiple lymphomatous polyposis; and colorectal cancer or adenoma in six patients. However, the autopsy findings in patients with both diseases were mostly pseudomembrane formation or ulcers due to fungal and/or bacterial infection. It is concluded that accurate endoscopic diagnosis of lower intestinal lesions in patients with leukaemia or malignant lymphoma is essential for staging and treatment of these diseases and for determining their prognosis. Most lesions in leukaemia are aphthoid and small ulcers are due to leukaemic infiltration or antibiotics; most lesions in malignant lymphoma are elevated lesions such as cancer, adenoma or lymphomatous lesions as determined by endoscopy. This is in contrast to pseudomembrane formation or ulcers due to fungal and/or bacterial infection which are detected at autopsy.     

Leukemic manifestation in a case of alpha-chain disease with multiple polypoid intestinal lymphocytic lymphoma.

We describe a female aborigine from Taiwan with alpha-chain disease associated with multiple polypoid intestinal lymphocytic lymphoma and leukemic manifestation. Initially, the patient experienced intermittent diarrhea, abdominal pain, and leukemic manifestation. No evidence of bone marrow involvement was found. Alpha-chain protein was demonstrated in the serum. Gastroendoscopy and a series of radiographs of the small intestine revealed multiple polypoid tumors involving the entire length of the small intestine. Duodenal biopsy showed diffuse lymphocytic lymphoma. Immunohistochemical staining of tumor samples revealed features typical of alpha-chain disease. Cytogenetic analysis showed the same abnormal karyotypes of neoplastic clones in intestinal tumor cells and in circulating leukemic cells. The data suggest that alpha-chain disease can present initially with intestinal multiple polypoid lymphocytic lymphoma and leukemic manifestation without evidence of bone marrow involvement. The data also support the homing theory of lymphomas from mucosa-associated lymphoid tissue.     

Human immunodeficiency virus infection of enterocytes and mononuclear cells in human jejunal mucosa

Intestinal malabsorption is a recognized cause of malnutrition in patients infected with human immunodeficiency virus. However, the relationships among human immunodeficiency virus infection, morphological changes in the intestine, and development of intestinal malabsorption are not well established. Nine patients infected with human immunodeficiency virus underwent tests of intestinal absorption and jejunal biopsies for morphometric measurements, enzyme assays, and virus detection by in situ hybridization. Steatorrhea and low lactase activities were found in more than 85% of the patients. All biopsy specimens were abnormal with reversal of the ratio of villus length to crypt depth in seven and enlarged enterocyte nuclear size in nine. Human immunodeficiency virus was detected in five jejunal biopsy specimens, within villus enterocytes of one patient who had the most severe malabsorption of the group and in four other biopsy specimens in mononuclear infiltrating cells of the lamina propria. These results suggest that human immunodeficiency virus infection of the small intestinal mucosa is an early event that is associated with altered enterocyte differentiation and function.     

原发性肠淋巴瘤与克罗恩病的鉴别诊断

背景：原发性肠淋巴瘤的发病率较低，临床表现缺乏特异性，易误诊为其他胃肠道疾病，特别是与克罗恩病（CD）的鉴别诊断较困难。目的：通过分析原发性肠淋巴瘤和CD患者的临床资料，提高原发性肠淋巴瘤与CD的鉴别诊断水平。方法：回顾性分析原发性肠淋巴瘤和CD患者的临床特点、内镜表现和病理检查结果。结果：原发性肠淋巴瘤患者的平均年龄为50岁，CD为35岁。5例（12.2％）CD发生肛周病变，4例（9.8％）内瘘，3例（7.3％）有肠外表现，但仅1例（5.9％）原发性肠淋巴瘤曾有肛瘘。内镜下原发性肠淋巴瘤以回盲部受累多见（41.7％），CD以小肠和结肠均受累多见（48，8％）；原发性肠淋巴瘤以肿块型较多见（41.7％），CD则主要表现为溃疡和铺路石样改变，其中18例伴肠腔狭窄，4例瘘管形成。B细胞性原发性肠淋巴瘤13例，T细胞性4例。12例CD表现为非干酪样坏死性肉芽肿。结论：临床上原发性肠淋巴瘤与CD的鉴别诊断较困难，需综合各种检查手段，特别是内镜检查和内镜下对病变部位多点取活检，以提高诊断率。     

Magnetic resonance imaging of bone marrow in lymphoproliferative disorders: correlation with bone marrow biopsy

In a prospective clinical study 10 normal volunteers and 30 patients with lymphoma--11 patients with Hodgkin's lymphoma and 19 patients with non-Hodgkin's lymphoma (11 low grade, 8 high grade)--were examined. Proximal femora, pelvis and lumbar spine were imaged with a 1.5 tesla superconducting MR imaging system (Siemens Magnetom). Areas of malignant infiltration in the bone marrow were clearly detected by visual and/or quantitative assessment. In most cases bone marrow involvement was demonstrated by both magnetic resonance imaging and bone marrow biopsies. However, in three of 30 patients magnetic resonance imaging showed evidence of lymphomatous involvement despite normal bone marrow histology. In one patient bone marrow cytology revealed malignant infiltration in the absence of MRI abnormalities. Thus, MRI is a sensitive method for detecting bone marrow infiltration by lymphoma.     

Radiological demonstration of colonic aphthoid ulcers in a patient with intestinal tuberculosis

The case is described of a young Asian woman with massive rectal haemorrhage during and after pregnancy. Barium radiology showed aphthoid ulcers in the colon and changes in the ileum suggesting tuberculosis. Colonoscopy revealed hyperplastic ulceration in the terminal ileum and culture of biopsies from this area grew Mycobacterium tuberculosis. The patient made a full and rapid recovery on anti-tuberculous therapy. Colonic aphthoid ulceration has not previously been recorded, radiologically, in intestinal tuberculosis.     

Idiopathic ulcer of the small bowel containing numerous plasma cells: case resembling mucosa-associated lymphoid tissue lymphoma

Idiopathic ulcer of the small bowel is a poorly recognized entity. Herein is reported a case of idiopathic ulcer of the jejunum containing numerous lymphoplasmacytoid infiltrates and mimicking mucosa-associated lymphoid tissue (MALT) lymphoma. An 82-year-old Japanese woman presented with nausea and vomiting. Following diagnosis of submucosal tumor, partial jejunal resection was performed. Macroscopically the resected specimen contained multiple small shallow ulcers. Histologically the lesion was composed of numerous plasma cells mixed with lymphocytes and histiocytes. Immunohistological study revealed that the plasma cells contained polytypic intracytoplasmic immunoglobulins. The patient remained free of disease after 36 months. Marginal zone B-cell lymphoma of MALT-type arising from small intestine occasionally shows prominent plasma cell differentiation. The present case demonstrates that idiopathic ulcer of the small bowel should be added to the differential diagnosis of MALT-type lymphoma of the small bowel.     

Recurrent colonic ulcers in celiac sprue,an unrecognized fatal complication: Report of a case

Summary In a patient who had celic sprue intractable to a gluten-free diet, volvulus and jejunal and colonic ulcers developed. Following segmental colonic resection, these ulcers recurred at the site of the previous anastomosis, and proved a fatal complication because of colonic perforation. Since no obvious etiology of colonic ulcers could be identified in this case, it is possible that they may represent an unrecognized intestinal complication of celiac sprue similar to jejunoileal ulcers.     

A case of adenocarcinoma developed in the small intestine with chronic strongyloidiasis

We experienced a case of intestinal strongyloidiasis complicated by jejunal carcinoma. A Japanese male in his 50s, who has a 7-year medical history of duodenal ulcers, complained of loss of appetite, nausea, vomiting and diarrhea. Computed tomography and gastroduodenal endoscopic examination revealed a stenosis of the duodenum. To remove the stenosis, gastric bypass surgery was performed. The pathological diagnosis of the resected jejunum was strongyloidiasis and well-differentiated adenocarcinoma with subserosal invasion and vascular infiltration. After administration of Ivermectin, Strongyloides stercoralis was not found in any biopsies or in the specimens of the intestine, which were resected due to cancer recurrence 2 years later. There are three possibilities for the reason of coexistence of S. stercoralis and adenocarcinoma: S. stercoralis caused the adenocarcinoma, S. stercoralis moved to the carcinoma, or just coincidence. Although it is difficult to prove a causal relationship between S. stercoralis and adenocarcinoma, this is the first report of adenocarcinoma developed in the jejunum with chronic strongyloidiasis. The number of nematode infections, including strongyloidiasis, is decreasing in Japan, although not worldwide. Therefore, it should be considered in patients with prolonged intestinal ulcers.     

Primary colorectal lymphoma comprising both components of diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma combined with cytomegalovirus colitis

A 16-year-old girl presented to our hospital with diarrhea and abdominal pain. The macroscopic findings of colonoscopy revealed multiple submucosal tumors and multiple ulcers, which were localized in the sigmoid colon, and diffuse granular mucosa which extended to the total colon. The pathological diagnosis was malignant lymphoma comprising both components of diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma, because the large lymphoma cells were CD20+, CD10?, and CD5?. Furthermore, immunohistochemical analysis of colorectal biopsy samples from multiple ulcers revealed cytomegalovirus (CMV)-positive cells. The patient was diagnosed with primary colorectal lymphoma comprising both components of DLBCL and MALT lymphoma combined with CMV colitis. She received anti-viral medication and chemotherapy.     

Virus-associated hemophagocytic syndrome and hemorrhagic jejunal ulcer caused by cytomegalovirus infection in a non-compromised host; a case report of unusual entity

A 76-year-old man was admitted to our hospital with abdominal pain, nausea, and vomiting. The patient was diagnosed as ileus by abdominal radiography, which showed an enlarged bowel and an air-fluid level. Computed tomography of the abdomen showed a thickened intestinal wall. His general status suddenly worsened, and he was placed on a respirator and catecholamines to prevent acute respiratory distress syndrome, septic shock, and disseminated intravascular coagulation. He had continuous fresh anal bleeding. Total colonoscopy showed bloody stool originating from the ileum. Emergency operation was performed for hemorrhagic shock under general anesthesia. Intraoperative jejunal endoscopy revealed deep linear ulcers with bleeding in the jejunum, and 30 cm of the jejunum was resected. Histopathologic examination revealed cytomegalic cells with intranuclear inclusion bodies in the tissues surrounding the ulcers, and it was diagnosed as cytomegaloviral enterocolitis with hemophagocytic syndrome in a non-compromised adult.     

Small intestinal injury and parasitic diseases in AIDS

OBJECTIVES: To examine jejunal biopsies from patients with the acquired immunodeficiency syndrome (AIDS), chronic diarrhea, and weight loss, and to correlate the presence of small intestinal injury with pathogens, histopathologic changes, and absorption. DESIGN: Prospective analysis of jejunal biopsies from 43 patients with AIDS, 10 patients with AIDS-related complex, and 6 heterosexual control volunteers. The biopsies were examined for pathogens; electron microscopy was used in 22 patients. The results of quantitative histologic measurements were correlated with histopathologic features and D-xylose absorption. SETTING: A university-affiliated teaching hospital with many AIDS patients of varying demographic characteristics. MEASUREMENTS AND MAIN RESULTS: Jejunal biopsies in 62% of the patients with AIDS had partial villus atrophy with or without crypt hyperplasia unlike those of controls, a result implying small intestinal injury. Small intestinal injury was associated with lymphoplasmacytic infiltration and cytopathic change of the villus epithelium. Light and electron microscopic examination detected cryptosporidia or microsporidia in 19 of 27 patients with small intestinal injury. The presence of these parasites was associated with significantly diminished D-xylose absorption when compared with absorption in controls or in patients with AIDS, diarrhea, weight loss, and no parasites. CONCLUSION: A significant proportion of patients with AIDS with severe small intestinal injury have enterocyte infections. Like cryptosporidiosis, intestinal microsporidiosis may be an important cause of enteropathy in patients with AIDS.