Crohn's disease and intestinal carcinoma. A report of 11 cases with emphasis on associated epithelial dysplasia.

Eleven patients with intestinal carcinoma complicating Crohn's disease are reported. In the 4 patients with small intestinal carcinoma, carcinomas were poorly differentiated or signet-ring cell type in 3 cases and mucinous type in 1 case. All 4 patients had high-grade dysplasia in the mucosa immediately adjacent to the carcinoma only. In the 7 patients with colonic carcinoma, tumors were mucinous type in 3, signet-ring cell type in 1, and well or moderately differentiated in 3. Dysplasia could be assessed in 6 patients. In 4 patients it was both adjacent to and distant from the invasive carcinoma. This distant dysplasia was focal in 2 cases. One patient showed dysplasia adjacent to the carcinoma only and in 1 other patient no dysplasia was seen. In a comparison group, all 7 cases of carcinoma associated with ulcerative colitis showed dysplasia adjacent to and distant from the invasive carcinomas. The authors conclude that (a) associated dysplasia is prevalent enough in Crohn's disease-associated carcinomas to support a dysplasia-carcinoma sequence and (b) dysplasia is more limited with carcinoma in Crohn's disease than ulcerative colitis, and this may make dysplasia less useful as a cancer surveillance marker in Crohn's disease.

Bile duct carcinoma in patients with ulcerative colitis. Relationship to sclerosing cholangitis: report of six cases and review of the literature.

Six cases of bile duct carcinoma were encountered among 1207 patients with ulcerative colitis, a prevalence rate of 0.5%. The relative risk of bile duct carcinoma in patients with ulcerative colitis was 31.3. Colitis was extensive in all six patients with a mean duration of 23.2 years before the diagnosis of carcinoma. The mean age at the diagnosis of carcinoma was 38.5 years. Three patients had undergone colectomy 5-16 years earlier, and in four patients pericholangitis and sclerosing cholangitis preexisted. The tumors, histologically adenocarcinomas, were located in the common bile duct in five patients and in the hepatic duct in one. The mean survival was 11.8 months (one patient is still alive with recurrent carcinoma). Pericholangitis and sclerosing cholangitis is a frequent preexisting lesion in patients with bile duct carcinoma complicating ulcerative colitis and may be considered a premalignant lesion in these patients. Both sclerosing cholangitis and bile duct carcinoma are rare in Crohn's disease.

Liver abscess in Crohn's disease. Report of four cases and review of the literature.

Four patients with liver abscesses and Crohn's disease are described, and reports of 14 cases in the English language literature are reviewed. The incidence of liver abscess in patients with Crohn's disease (114-297 per 100,000) appears to be higher than that of liver abscess in the general population (8-16 per 100,000). Frequently the clinical manifestations of liver abscess are mistaken for a reactivation of Crohn's disease, and diagnosis is delayed. In comparison to patients with liver abscess in the general population, patients with Crohn's disease and liver abscess are considerably younger, are more likely to have multiple rather than solitary abscesses, and usually have a predisposing intraabdominal focus of infection, rather than a biliary one. Streptococci, especially Streptococcus milleri, are the most frequent cause of liver abscess in patients with Crohn's disease. Liver scanning should be performed routinely in patients with Crohn's disease in whom a febrile illness cannot be completely explained by bowel disease, or in whom fever does not respond to drainage of intraabdominal abscesses.

Colorectal and extracolonic malignancy in ulcerative colitis.

In a review of 1248 cases of ulcerative colitis seen at the Cleveland Clinic that were followed up to 1984 (mean, 14.4 years), 82 patients (6.5%) were subsequently found to have colorectal cancer and 48 (3.8%) had extracolonic malignancy, 6 of them with associated colorectal cancer. Most patients with colorectal cancer were men (2:1), and had extensive (90%) and long-lasting colitis (10 years or more in 93% of cases; mean 18 years). Colitis was inactive before the diagnosis of cancer in 48%. Acute onset of the first attack was rare (7%), and the disease had a remittent course in 92%. The mean age at diagnosis of cancer was 43 years. The cumulative risk of colorectal cancer was significantly higher in patients with extensive colitis than in those with left-sided disease (P less than 0.0001: 11.9% vs. 1.8% at 20 years and 25.3% vs. 3.7% at 30 years). When comparing mean duration of disease, left-sided colitis (22 years) did not differ significantly from extensive disease. The tumor was multifocal in 13.5%, proximal to the splenic flexure in 44%, and poorly differentiated in 34% of the cases. The diagnosis was suspected clinically in 64% of cases. The prognosis of colorectal cancer in patients with ulcerative colitis appears to be similar to that in the general population. The cumulative 5-year survival rate was 54%. This study supports the concept that surveillance colonoscopy should be started after 8 to 10 years of extensive colitis and after 15 years of left-sided colitis. Among those with extracolonic malignancy, the incidence of bile duct carcinoma, leukemia, bone tumors, and endometrial cancer was significantly greater than expected (P less than 0.01), whereas that of lung cancer was significantly lower than expected (P less than 0.01).

Inflammatory bowel disease and leukemia. A report of seven cases of leukemia in ulcerative colitis and Crohn's disease and review of the literature.

In a review of a large number of patients with inflammatory bowel disease, leukemia was observed in five patients with chronic ulcerative colitis and in two patients with Crohn's disease. In ulcerative colitis patients, there were three cases of acute myelocytic leukemia and one case each of acute lymphoblastic leukemia and chronic granulocytic leukemia. In Crohn's disease patients, there was one case each of chronic granulocytic leukemia and chronic lymphocytic leukemia associated with thrombocythemia. Sixteen other cases of leukemia have been reported to date in inflammatory bowel disease. All types of leukemia, but particularly acute myelocytic leukemia, have been described. There has been no single common feature as to type (whether ulcerative colitis or Crohn's disease), extent and course, or medical and surgical treatment of the bowel disease. The relative risk of leukemia in patients with ulcerative colitis was 5.3 [95% confidence interval 1.7 to 12.3 (P less than 0.01)] and of acute myelocytic leukemia 11.4 [95% confidence interval 2.3 to 24.9 (P less than 0.01)]. Our data on patients with Crohn's disease were not sufficient to assess the statistical significance of leukemia in this disease. This study suggests that there may be an increased risk of leukemia, particularly acute myelocytic leukemia, in ulcerative colitis. The causal relationship, if any, remains undetermined.

Course and prognosis of idiopathic ulcerative proctosigmoiditis in young patients.

The course and prognosis of idiopathic ulcerative proctosigmoiditis were studied in 85 young patients whose symptoms had begun before the age of 21 (mean age at onset, 16 years), and the results were compared with those in adults with proctosigmoiditis. Data regarding extension of disease, available in 66 patients, allowed us to identify two groups of patients. Presenting symptoms were the same in both groups. In Group I (41 patients, 62%), the disease remained stable or did not extend beyond the descending colon. In Group II (25 patients, 38%), there was evidence of extension to the hepatic flexure or beyond. Extension was unpredictable in individual patients, but generally occurred within 5 years from the onset of symptoms (73%). The clinical course and prognosis were different in each group. In Group I, there were more females, the disease ran a milder course, and there were few complications. In Group II, there were relatively more males, the disease was more active and severe, and a high incidence of intestinal and extraintestinal complications was observed. Fifteen patients, all from Group II, required colectomy. The natural history of proctosigmoiditis in young patients is somewhat different from that in adults, being characterized by a greater tendency to proximal extension (38% vs. 10%). However, when the disease remains confined to the rectosigmoid region (or does not spread beyond the splenic flexure), the course and prognosis are no different than in adults.

Hypereosinophilia, ulcerative colitis, sclerosing cholangitis, and bile duct carcinoma.

A case of ulcerative colitis and pericholangitis-sclerosing cholangitis bile duct carcinoma complex associated with hypereosinophilia and possibly the hypereosinophilic syndrome is reported, and the association of eosinophilia with various hepatobiliary and gastrointestinal diseases is discussed. Hypereosinophilia may foretell a more serious underlying condition such as bile duct carcinoma in some patients with primary sclerosing cholangitis.

Ulcerative colitis in Iran: a review of 112 cases.

Despite claims of rarity, chronic ulcerative colitis is being encountered with increasing frequency in developing countries. We report our experience with 112 patients with chronic ulcerative colitis diagnosed during a 10-year period between 1973 and 1982 in Tehran, Iran, and compare the demographic and clinical features of chronic ulcerative colitis on our patients with those reported from other countries. Significant differences included the mild course of the disease, the absence of skin manifestations, and the rarity of colorectal cancer in our patients. The importance of changing socioeconomic status and cultural factors on the development of chronic ulcerative colitis is briefly discussed. The extreme rarity of Crohn's disease in Iran is noted.

Primary tuberculous granulomatous esophagogastro-duodenitis: a report of a case.

Primary upper gastro-intestinal tuberculosis is a very rare disease. We report the case of a 25-year-old man who presented with a febrile illness and non-specific gastro-intestinal symptoms. The upper gastro-intestinal X-rays showed a normal esophagus and non-specific changes of the stomach and duodenum. Endoscopy revealed diffuse esophagogastro-duodenitis of tuberculous origin. The patient recovered completely after treatment.

Clinical course and evolution of erythema nodosum and pyoderma gangrenosum in chronic ulcerative colitis: a study of 42 patients.

The authors examined 1132 patients with chronic ulcerative colitis (CUC) seen at the Cleveland Clinic to clarify the relationship between the clinical features of colitis and the incidence and severity of erythema nodosum (EN) and pyoderma gangrenosum (PG). There were 21 patients (2%) with EN and 21 (2%) with PG, both of which affected those with active and extensive colitis. CUC was inactive in only 10% of the EN group and 20% of the PG group; it was left-sided in 20% of the EN group and 15% of the PG group. The mean duration of CUC before the appearance of EN and PG was 5 and 10 years, respectively. Three patients had consulted a dermatologist for PG before a clinical diagnosis of CUC was made. EN and PG recurred in 20 and 33% of cases, respectively. EN appeared almost exclusively on the legs, while PG appeared on various areas of the skin, including the site of ileostomy in one patient. EN was controlled with adequate medical or surgical treatment of CUC, but PG was relatively refractory, requiring specific treatment of its own in 30% of patients. In one case each, EN and PG recurred after subtotal colectomy but had resolved completely after proctectomy. In one patient, PG developed for the first time 1 year after total colectomy. Less than half of EN patients and two-thirds of PG patients ultimately had to undergo colectomy because of bowel disease. No patient required colectomy because of skin lesions.

Coexisting primary malignant lymphoma and adenocarcinoma of the large intestine in an IgA-deficient boy.

This is a case report of an IgA-deficient adolescent male (14 years old) presenting with coexisting adenocarcinoma and adenomatous polyp of the rectosigmoid, and a primary lymphoma of the cecum (large-cell, histiocytic type). A 14-year-old sister of the patient had died of gastric carcinoma. Her serum immunoglobulins were not measured. The literature regarding the coexisting primary malignant lymphoma and adenocarcinoma of the colon, as well as neoplasia associated with IgA deficiency, is reviewed and pertinent literature discussed.

Malakoplakia of the colon and recurrent colonic strictures in a patient with primary hypogammaglobulinemia: an association not previously described.

A nine-year old boy with primary hypogammaglobulinemia and recurrent colonic strictures who developed malakoplakia of the colon is reported on. Such an association has not been reported previously. The development of colonic strictures is not considered a gastrointestinal manifestation of hypogammaglobulinemia. Malakoplakia of the colon is a rare disease, with 26 cases being reported to date. Only five children under age 13 have been reported with colonic malakoplakia and none with hypogammaglobulinemia. Hypogammaglobulinemia should be added to the growing list of conditions predisposing to the development of malakoplakia.

Alpha-chain disease--a report of eleven patients from Iran.

About one hundred cases of alpha-chain disease have been reported to date from various Mediterranean and Middle-Eastern countries. We report eleven Iranian patients with primary lymphoma of the duodenum and upper small intestine and positive alpha-chain protein in their sera. The clinical, laboratory and pathological findings in these patients are discussed. The initial favourable response of some of these patients to antibiotic treatment is noted. It is concluded that the optimal treatment of alpha-chain disease is not yet well established, and the clinical improvement might not coincide with histological and immunological remission.