Small bowel adenocarcinoma presenting as a first manifestation of Crohn's disease: report of a case,and a literature review

Small bowel Crohn's disease has been related to an increased incidence of small bowel adenocarcinoma, but the total number of reported cases is small. We present an interesting case of a young male patient with nephrolithiasis in childhood, an atypical intermittent history of diarrhoea also since his childhood, who developed obstructive ileus and underwent an urgent operation. The operation revealed a stenosis of the ileum owing to a mass, which proved to be a small bowel adenocarcinoma. One month later, the patient underwent a curative surgical resection of the tumour with additional lymphadenectomy, followed by chemotherapy for 6 months. Since then, the patient had mild diarrhoea but enteroclysis was normal. Approximately 2 years after the resection of the tumour, a new ileocolonoscopy demonstrated deep ulcerations of the terminal ileum and the adjacent area of the colon as well as anal ulcerations. The new biopsy specimens were convincing for Crohn's disease. There were no signs of residual or relapsing cancer. There is growing evidence that Crohn's disease is one of the triggering factors for the development of small bowel adenocarcinoma. Underlying Crohn's disease should be suspected in a young patient with an atypical history of diarrhoea and small bowel adenocarcinoma.     

Primary intestinal lymphoma complicating Crohn's disease

A 32-year-old man with a 12-year history of Crohn's disease of the colon was found to have a 5-cm cecal mass on colonoscopy. Histology examination of the lesion revealed high-grade B-cell lymphoma of Burkitt's type. He was treated with chemotherapy and has done well in the past 12 months. Review of the literature reveals 30 cases of lymphoma in patients with a history of Crohn's disease.     

Ulcerative colon T-cell lymphoma: an unusual entity mimicking Crohn's disease and may be associated with fulminant hemophagocytosis

Primary gastrointestinal T-cell lymphoma is uncommon. Most arise from the small intestine and are usually associated with chronic celiac disease; the so-called enteropathy associated T-cell lymphoma. Primary colon T-cell lymphoma is much more rare. We present two patients with primary colon T-cell lymphoma. Both patients had chronic diarrhea and significant weight loss. Endoscopically, the lymphoma was characterized by the presence of multiple skipped ulcers distributed from the terminal ileum to the descending colon. It was differentiated from Crohn's disease by the absence of fistula or thickening of the intestinal walls. Histologically, the lymphoma was composed of medium to large atypical cells located in the ulcer base with extension to the muscular layer and the adjacent atrophic mucosa. Occasional increased intraepithelial lymphocytes were also seen. Immunohistochemically, the lymphoma cells and intraepithelial lymphocytes were CD3+, CD4-, CD56- and CD8-. It was difficult to diagnosis this unusual lymphoma by biopsy. Because most biopsy specimens showed mixed inflammation within which the lymphoma cell was sometimes hard to identify. Both patients died of fulminant hemophagocytic syndrome and Epstein-Barr virus genome was detected in the lymphoma cells using in situ hybridization on the final surgical specimens. Our study indicates that it is important to recognize this ulcerative colon T-cell lymphoma and to differentiate it from inflammatory bowel disease because of its much more aggressive clinical behavior.     

Tabulation of myeloid, lymphoid and intestinal malignancies in Crohn's disease.

A variety of malignant complications occur in Crohn's disease, and previous studies have recorded an increased intestinal cancer risk. The present investigation tabulated myeloid and lymphoid malignancies compared with intestinal cancers in 1000 consecutively evaluated patients with Crohn's disease who were followed over an extended period by a single clinician. Myeloid and lymphoid neoplasms were present in 0.5% of patients, while cancer in the intestinal tract was detected in 1%. Most of these patients with a malignancy had Crohn's disease for a prolonged period of more than 20 years and had negative outcomes, including death or presentations with advanced disease. In this cohort, lymphoma was not detected in a single patient after definition of Crohn's disease, possibly reflecting the limited use of immunosuppressives or infused biological agents in this clinical practice. Bypassed rectal 'stumps' were associated with subsequent colorectal cancer in half of all males with colon cancer in this series, suggesting an important risk factor following colectomy in Crohn's disease. Epithelial dysplasia was detected in only a single male patient before colorectal cancer, implying that this histopathological marker may be a poor predictor of subsequent colon cancer development in Crohn's disease, an inflammatory bowel disease process that is typically patchy or focal in distribution in the intestinal tract.     

Evaluation of criteria for the activity of Crohn's disease by power Doppler sonography

BACKGROUND/AIM: In recent years, power Doppler sonography has been proposed as a method to assess disease activity in patients with Crohn's disease. The aim of this prospective study was to evaluate diagnostic criteria for power Doppler sonography by blinded comparison with ileocolonoscopy. METHODS: Twenty-two patients with confirmed Crohn's disease were prospectively investigated with B-mode and power Doppler sonography (HDI 5000, Philips Ultrasound) as well as ileocolonoscopy. Sonography was performed within 3 days before endoscopy. All procedures were performed by experienced examiners who were blinded to the clinical data and other results. Defined ultrasound parameters (bowel wall thickness, vascularization pattern) were used to determine a sonographic score of the activity. The degree of activity was scored from 1 (none) to 4 (high) by both ultrasound and ileocolonoscopy (pattern, extent of typical lesions). For each patient all segments of the colon and the terminal ileum were evaluated by both ultrasound and endoscopy. The weighted kappa test was used (StatXact software) for statistical analysis. RESULTS: In total, 126 bowel segments were evaluated by both ultrasound and endoscopy. The study showed a high concordance of power Doppler sonography and ileocolonoscopy (weighted kappa by region: sigmoid colon: 0.81; transverse colon: 0.78; ascending colon: 0.75; cecum: 0.84; terminal ileum: 0.82). Highest concordance was found in the descending colon (weighted kappa: 0.91; 95% CI: 0.83-0.98). CONCLUSIONS: Combination of B-mode and power Doppler sonography has a high accuracy in the determination of disease activity in Crohn's disease when compared to ileocolonoscopy. The diagnostic criteria established in this study can be useful for the evaluation of inflammatory bowel diseases by ultrasound.     

Crohn's Ileitis in a Patient with Longstanding HIV Infection

The differential diagnosis of diarrhea in patients infected with HIV is broad, and includes a variety of bacterial, viral, and parasitic pathogens, as well as malignancies including lymphoma and Kaposi's sarcoma. Idiopathic non-specific inflammatory bowel disease rarely occurs in association with HIV infection. A recent case report described a patient with longstanding Crohn's disease who experienced remission of his bowel disease upon infection with HIV (6). The authors inferred that the remission was secondary to a depressed CD4 (T helper) lymphocyte count. We report the first case of Crohn's ileitis developing in a patient with established HIV infection, depressed CD4 lymphocyte count, and no prior history of inflammatory bowel disease. This case raises questions about the role of CD4 cells in the pathogenesis of Crohn's disease.     

Duodenal involvement with Crohn's disease: a spectrum of radiographic abnormality.

The clinical and radiographic features of 22 cases of duodenal Crohn's disease were analyzed. The presenting clinical findings in the majority suggested peptic disease rather than regional enteritis. There was no cases of isolated duodenal Crohn's disease but a spectrum of radiographic abnormalities was produced by duodenal involvement with Crohn's disease which simulated a variety of clinical entities. A radiographic examination of the small bowel or colon was useful to confirm a diagnosis of Crohn's disease when duodenal abnormalities were suggestive.     

Abnormal intestinal permeability pattern in colonic Crohn's disease. Absorption of low molecular weight polyethylene glycols after oral or colonic load

Intestinal permeability to different-sized polyethylene glycols in Crohn's disease of the colon was compared with that in ileal Crohn's disease and in controls without inflammatory bowel affection. The permeability was assessed both after ingestion of the marker (oral load) and after deposition in the colon during colonoscopy (colonic load). After oral load the absorption was least in the patients with colonic Crohn's disease, intermediate in ileal disease, and greatest in the controls. After colonic load, however, the values were highest in colonic Crohn's disease. The study indicated that in Crohn's disease of the colon there is abnormal permeability in apparently uninvolved proximal small intestine as well as in the colon. Since oral load tests preferentially reflect the absorptive properties of the proximal small bowel, regional tests of absorption are important when the aim is to assess the permeability of the distal small intestine or the colon.     

Peptide YY concentrations in normal ileum and colon and in idiopathic inflammatory bowel disease

Concentrations of the candidate endocrine and paracrine peptide, peptide YY, were measured by specific radioimmunoassay in tissue extracts prepared from normal ileum, normal colon, Crohn's disease, and ulcerative colitis. In both the ascending and descending colon, there were significantly decreased mean concentrations of peptide YY in Crohn's colitis and ulcerative colitis, compared to mean concentrations in normal colon. There was no age-related decrease of concentrations of peptide YY in normal colon. The decrease in concentrations of peptide YY in colon obtained from patients with inflammatory bowel disease did not appear related either to the duration of the clinical symptoms of the disease or to the severity of colonic inflammation. Further studies of the physiological function of peptide YY in man are needed to determine whether these findings might be useful in understanding a component of the pathophysiology of idiopathic inflammatory bowel disease.     

Cytomegalovirus infection in a patient with Crohn's ileocolitis.

Cytomegaloviral enterocolitis is an uncommon infection that can complicate inflammatory bowel disease. A case of a patient with a three-year history of Crohn's disease is reported. He had been in a stable condition on mesalamine 4 g/day and methylprednisolone 10 mg/day for three years until four weeks before admission. The patient was admitted with complaints of fever, abdominal pain and watery diarrhea. A diagnosis of an exacerbation of Crohn's disease was established. The radiological examination revealed narrowing of the terminal ileum. Multiple fistulas and abscess-like images were observed. The patient then underwent ileocolic resection and ileostomy. The histopathological examination revealed Crohn's ileocolitis with superimposed cytomegalovirus infection. In patients with rapidly deteriorating inflammatory bowel disease, cytomegalovirus infection should be kept in mind as one of the differential diagnoses.     

Utility of a Rapid Fecal Latex Agglutination Test Detecting the Neutrophil Protein, Lactoferrin, for Diagnosing Inflammatory Causes of Chronic Diarrhea

Objective: The utility of tests for fecal neutrophils in the setting of chronic diarrhea has not been established. The purpose of this study was to determine the causes of chronic diarrhea associated with fecal neutrophils.
Methods: One fecal specimen from each of 10 normal subjects, 26 patients with known microscopic colitis, 13 with celiac sprue, eight with Crohn's disease, four with ulcerative colitis, and 103 with chronic diarrhea of unknown origin, as well as 10 fecal specimens from a patient with chronic nongranulomatous enterocolitis were analyzed blindly for the presence of a neutrophil granule protein called lactoferrin using a commercial latex agglutination kit. Diagnostic evaluation of the 103 patients with chronic diarrhea was carried out to determine the diagnostic accuracy of this test for chronic inflammatory bowel disease.
Results: None of the normal control subjects, three of 39 patients with microscopic colitis or celiac sprue, all 10 specimens from the patient with enterocolitis, and all 12 control patients with ulcerative colitis or Crohn's disease had a positive fecal lactoferrin test. Eleven of 103 patients with chronic diarrhea presenting without a diagnosis had a positive test, and all were diagnosed with an inflammatory condition of the colon (five-, ulcerative colitis; four-, Crohn's disease; one-, ischemic colitis; and one-, microscopic colitis). Only one patient with inflammatory bowel disease had a negative lactoferrin test. The sensitivity, specificity, and positive and negative predictive values of the fecal lactoferrin test for ulcerative or Crohn's colitis were 90%, 98%, 82%, and 99%, respectively.
Conclusion: The major cause of fecal neutrophils in patients with chronic diarrhea is chronic inflammatory bowel disease of the colon. The latex agglutination test for fecal lactoferrin offers a highly sensitive, specific, and simple means for detection of fecal neutrophils in these patients.     

Rheumatoid vasculitis with multiple intestinal ulcerations: report of a case]

Rheumatoid vasculitis is a relatively uncommon complication of rheumatoid arthritis (RA). It shows wide variety of extra-articular features including skin and neurologic involvement, but rarely shows gastrointestinal involvement. We describe a 79-old-man of rheumatoid vasculitis manifesting as small bowel multiple ulcerations accompanied by perforation of the descending colon diverticulum. The patient was admitted to our hospital with rectal bleeding and severe anemia. He had had rheumatoid arthritis for more than 10 years with treatment of non-steroidal anti-inflammatory drugs (NSAIDs) and low-dose prednisone. Upper endoscopy or colonoscopy revealed no evidence of bleeding. Suddenly he developed cramping abdominal pain, and emergency operation was performed. Segments of the descending colon and small bowel were resected to reveal perforation of the descending diverticulum and severe multiple ulcer of small bowel. The pathological examination showed multiple ulcerations of the small intestine was caused by vasculitis.     

A case of Behcet's disease with intestinal involvement due to Crohn's disease

A 15-yr-old, Arabic male presented with painful, recurrent, self-resolving oral and genital ulcers, erythema nodosum and uveitis. Behcet's disease was diagnosed. A few months later, he experienced anorexia, abdominal pain, diarrhea, and weight loss. Although all routine laboratory and radiologic investigations were negative, colonoscopy revealed the presence of serpiginous ulcers with pseudopolyps and inflamed intervening mucosa in the proximal half of the colon. Therapy with oral steroids was helpful, but the disease exacerbated a few months after prednisone was discontinued. Repeat evaluation showed similar endoscopic findings and, on colonic biopsy, noncaseating granulomas compatible with Crohn's disease were seen. Again, the patient responded well to oral steroids and sulfasalazine. We believe that gastrointestinal involvement in our patient is compatible with Crohn's disease and that screening tests to rule out chronic inflammatory bowel disease should be performed in the presence of gastrointestinal involvement in Behcet's disease. Behcet's disease may be a part of the spectrum of chronic inflammatory bowel disease.     

Somatostatin in the idiopathic inflammatory bowel diseases

To study the effect of mucosal inflammation on tissue concentrations of somatostatin, the distribution and concentration of somatostatin in specimens of normal and abnormal (ulcerative colitis and Crohn's disease) ileum and colon were determined by a specific radioimmunoasay. Each tissue specimen obtained at surgery was separated by microdissection into the mucosa-submucosa and the muscularis externa. Immunoreactive somatostatin was acid-extracted from each layer before measurement. Gel chromatography was used to characterize immunoreactive somatostatin measured by radioimmunoassay; somatostatin-28 was the major immunoreactive species measured in human intestine. In normal colon, concentrations of somatostatin were not related to patient age. Concentrations of immunoreactive somatostatin in the mucosa-submucosa of the descending colon were significantly decreased in ulcerative colitis and in Crohn's colitis, compared with normal colon. There was no apparent relationship between concentrations of somatostatin and the duration of inflammatory bowel disease. However, somatostatin concentrations appeared to be lower in patients with severe colitis than in patients with minimal colitis. The decrease in mucosal-submucosal concentrations of somatostatin is in agreement with previous morphologic studies, which have suggested diminished populations of endocrine cells in ulcerative colitis. The possible role of somatostatin in the colon suggests that further studies of the alteration of this gut peptide may be useful in understanding a component of the pathophysiology of idiopathic inflammatory bowel disease. Dr. Koch is a recipient of a Career Development Award from the National Foundation for Ileitis and Colitis.     

Crohn disease. Results of surgical treatment

The aim of this study was to describe and to analyze the results of surgery for Crohn's disease in a retrospective series of 155 operations performed in 124 patients from 1949 to 1981 by the same surgical team. Indications for surgical treatment were as follows: acute complications (25 p. 100), intestinal obstruction (35 p. 100), systemic inflammatory manifestations (40 p. 100). Three patients died postoperatively and complications necessitated a reintervention in 10 other subjects. Surgical indications and late postoperative prognosis were significantly different in patients with right-sided bowel lesions (i. e. terminal ileitis, ileocolitis of the ascending colon) and in those with left colonic involvement (i.e. colitis of the whole or descending colon, rectitis and anoperineal lesions). Surgery was required because of failure of medical treatment in 73 p. 100 of Crohn's disease affecting the left colon and in 17 p. 100 of the right-sided lesions (p less than 0.001). After resection a recurrence rate after 1 year and 3 years of respectively 50 and 62 p. 100 was observed in the left colonic disease group and in 5 and 23 p. 100 of the cases respecting the left colon. Finally 31 reoperations were necessary; no surgical mortality occurred in 12 patients with right-sided lesions whereas 5 among 19 patients with involvement of the left colon died postoperatively. These results: a) confirm the high postoperative rate of recurrence (or relapse) after surgical bowel resection for Crohn's disease (especially when the left colon is affected), b) emphasize the need for the surgeon to be very cautious when determining the extent of resection.     

Crohn's Disease presenting as Isolated Jejunitis Years after Childhood Colitis

We present a case of a patient who had documented ulcerative colitis as a child and later presented with isolated Crohn's jejunitis. Although rare, Crohn's disease must be considered in those patients with segmental inflammation of the small bowel and a prior history of inflammatory bowel disease involving the colon. Patients with colitis that do not show specific criteria for either ulcerative colitis or Crohn's disease should be classified as indeterminant colitis, and a small bowel series is indicated. It may be indicated to obtain a small bowel series in patients with any form of colonic inflammatory bowel disease, periodically, after diagnosis.     

Risk of lymphoma in inflammatory bowel disease

OBJECTIVE: Inflammatory bowel disease with a subsequent diagnosis of non-Hodgkin's lymphoma has been reported. There is concern that the risk of developing lymphoma will rise with increasing use of immune modifier therapy. We determined the risk of non-Hodgkin's lymphoma in an U.S. population-based inception cohort, and evaluated the association between inflammatory bowel disease and lymphoma in our referral practice. METHODS: The records of all incidence cases of inflammatory bowel disease in Olmsted County, Minnesota, between 1950 and 1993 were reviewed for the diagnosis of lymphoma. Standardized incidence ratios (observed/expected) were used to estimate relative risk. In addition, the clinical features and outcomes of all patients with inflammatory bowel disease and lymphoma seen at Mayo Clinic between 1976 and 1997 were reviewed. RESULTS: Among 454 county residents diagnosed with inflammatory bowel disease, a single non-Hodgkin's lymphoma occurred in a patient with Crohn's disease. No cases were seen with ulcerative colitis. The estimated relative risk of lymphoma was 2.4 in Crohn's disease (95% confidence interval, 0.1-13), 0 in ulcerative colitis (0-6), and 1.0 in inflammatory bowel disease overall (0.03-6). Between 1976 and June 1997, 61 patients with inflammatory bowel disease and lymphoma (approximately 0.41%) were seen in the referral practice. In four patients with Crohn's disease (13%), potential neoplastic risk factors were identified-therapeutic radiation in 1, and use of purine analogs in 3 (median length of use, 11 months). CONCLUSIONS: Our population-based cohort study demonstrated that the absolute risk of non-Hodgkin's lymphoma remains quite small (0.01% per person-year). This risk may not exceed that in the general population. In our referral practice, immune modifier therapy could be potentially implicated in only 5% of cases of lymphoma occurring in the setting of inflammatory bowel disease.     

Virtual colonoscopy-induced perforation in a patient with Crohn＇s disease

We report a case of sigmoid colon perforation in a patient with Crohn's disease undergoing computed-tomographic (CT) colonography. A 70-year-old patient with Crohn's disease with terminal ileitis and sigmoid stricture underwent CT colonography after incomplete conventional colonoscopy. During the procedure, the colon was inflated by air insufflation and the patient developed abdominal pain with radiological evidence of retroperitoneal and intraperitoneal free gas. Hartmann's operation was performed. This case highlights that CT colonography is not risk-free. The risk of perforation may be higher in patients with inflammatory bowel disease.     

Perianal Hodgkin's lymphoma complicating Crohn's disease

BACKGROUND: The perianal region is a very rare location for Hodgkin's lymphoma, and clinicians may often neglect the diagnosis in patients with inflammatory bowel disease. PATIENT PRESENTATION: We present a case of perianal Hodgkin's lymphoma in patient with Crohn's disease who was on long-term immunosuppression and whose symptoms would normally be attributed to Crohn's disease. Diagnosis was based on the morphological appearance of atypical cells in the lamina propria and the immunohistochemical profile of Reed Sternberg and Hodgkin's cells, showing co-expression of CD15 and CD30. CONCLUSION: Perianal complaints in patients with inflammatory bowel disease may be a manifestation of other pathology. Hodgkin's lymphoma could be a progression in the chronically inflamed tissue in this unusual location.     

Colorectal cancer risk in Crohn＇s disease

There is recognized increased risk for colorectal cancer in patients with inflammatory bowel disease, particularly in long-standing and extensive ulcerative colitis. There also appears to be an increased rate of intestinal cancer in Crohn＇s disease, including both colon and small bowel sites. In Crohn＇s disease, evidence suggests that detection of colorectal cancer may be delayed with a worse prognosis. Some risk factors for cancer in Crohn＇s disease include the extent of inflammatory change within the colon and the presence of bypassed or excluded segments, inclu- ding rectal ＂stump＂ cancer. In addition, the risk for other types of intestinal neoplasms may be increased in Crohn＇s disease, including lymphoma and carcinoid tumors. Earlier detection of colorectal cancer based on colonoscopy screening and surveillance may be achieved but, to date, this has not translated into a positive survival benefit. Moreover, newer staining methods and evolving micro-endoscopic techniques show promise, but have not significantly altered management. Future research should focus on development of molecular or other bio-markers that might predict future dysplasia or cancer development in Crohn＇s disease.