Common variable hypogammaglobulinemia associated with intestinal lymphoid hyperplasia

A case of adult common variable hypogammaglobulinemia with nodular lymphoid hyperplasia characterized by malabsorption and enteric protein loss, probably due to bacterial overgrowth, is reported. This clinical condition is of particular interest because of the unusual pathology and the lack of an efficient treatment. The association between common variable hypogammaglobulinaemia and nodular lymphoid hyperplasia must be considered in young adults with recurrent respiratory tract infection, gastrointestinal symptoms, diarrhoea, hypogammaglobulinemia and low serum albumin.     

Nodular lymphoid hyperplasia with hypogammaglobulinaemia

A patient with nodular lymphoid hyperplasia, who manifested defects in cellular immunity, is presented. The clinical course is discussed and a review included of the 23 patients thus far reported with nodular lymphoid hyperplasia and hypogammaglobulinaemia.     

Nodular lymphoid hyperplasia of the small bowel associated with two primary colonic adenocarcinomas

A patient presented with rectal bleeding and weight loss. On barium enema two space-occupying lesions, one in the cecum and the other in the right transverse colon, were demonstrated. He underwent right hemicolectomy. The surgical specimen demostrated diffuse nodular lymphoid hyperplasia (NLH) of the small intestine and two primary carcinomas of the colon, an association that to the best of our knowledge has not been previously described. Received: 26 July 1999 / Accepted in revised form: 12 September 1999     

Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities

BACKGROUND/AIMS: Liver lesions associated with primary hypogammaglobulinemia have been poorly described. We aimed to assess the clinical, histological and immune features and outcome of hepatic injury in patients with primary hypogammaglobulinemia. METHODS: The medical records of 51 patients (23 patients with liver biopsy) with primary hypogammaglobulinemia and liver abnormalities were retrospectively reviewed. Forty-three controls with primary hypogammaglobulinemia but with no hepatic manifestations were analyzed in parallel. RESULTS: Cholestasis (65%), mainly anicteric, and portal hypertension (50%) were the main hepatic manifestations. Histological analysis revealed non-fibrosing architectural abnormalities consistent with nodular regenerative hyperplasia (NRH) in 84% of CVID patients and in all HIGM and XLA patients. Intrasinusoidal lymphocytic infiltration, abnormalities of portal vessels and epithelioid granulomas were observed in 90%, 43% and 44% of patients, respectively. NRH was associated with portal hypertension in 75% of the cases. These patients more often presented with autoimmune diseases and peripheral lymphocytic abnormalities than control patients (p < 0.05). CONCLUSIONS: Liver involvement in primary hypogammaglobulinemia mainly consists of NRH leading to chronic cholestasis and portal hypertension. Association with intrasinusoidal T cell infiltration, portal vein endotheliitis, autoimmune diseases and peripheral lymphocytic abnormalities suggests an autoimmune mechanism.     

Nodular lymphoid hyperplasia of the small intestine and malignant lymphoma

Digestive Diseases and Sciences -     

Nodular lymphoid hyperplasia of the duodenum in cancers of the gastrointestinal tract

In an autoptic study the coincidence of carcinomas of the gastrointestinal tract and nodular lymphoid hyperplasia (NLH) of the duodenum was studied histologically. Out of sixty cases of carcinomas in the gastrointestinal tract not one case of concomitant NLH in the duodenum could be demonstrated, nor in the first section of the jejunum, which was examined for comparison's sake. A connection between NLH and carcinomas of the gastrointestinal tract, as pointed out in the literature, could not be confirmed through this study.     

In vitro sensitization and mast cell degranulation in human jejunal mucosa.

Sera from 3 allergic patients with specific IgE antibodies as shown by RAST were used to sensitize jejunal mucosa obtained from surgical patients. The sensitized specimens were challenged with the appropriate antigens to the specific IgE shown in the sera, Non-challenged sensitized specimens were used as controls to determine mast cell degranulation. The mast cells were counted in a defined area in the mucosa immediately adjacent to the muscularis mucosa. Mast cell degranulation was 47 percent in a timothy pollen system, 40 percent in an eggwhite system, and 33 percent in a codfish system. The results of the investigation indicates that mast cells in the human jejunal mucosa are able to react in the same manner as mast cells in the human lung. The experimental model described appears suitable for studying the allergic reaction in the gastrointestinal tract and the effect of pharmacotherapy in this respect.     

Increased numbers of mast cells in bronchial mucosa after the late-phase asthmatic response to allergen.

We examined the characteristics of allergen-induced inflammation of the bronchial mucosa in asthmatic patients. Studies were carried out 4 h (eight patients) and 24 h (nine patients) after allergen inhalation challenge; 10 patients were not challenged and served as control subjects. We found that in the control group the ratio of degranulating to granulated mast cells was higher in patients with than in patients without late-phase response. In patients studied 4 h after allergen challenge the total number of mast cells was not significantly different from that in control subjects; the ratio of degranulating to granulated mast cells was increased similarly in patients with and without late-phase response. Among patients studied 24 h after allergen challenge, those who had developed the late-phase response had an increased (p less than 0.05) number of mast cells as compared with patients who had not developed the late-phase response, the number of mast cells was significantly correlated with the severity of the late-phase response (r = 0.80; p less than 0.001). The numbers of eosinophils and mononuclear cells and the morphologic abnormalities of bronchial structure (altered ratio of cylindrical to goblet cells, thickening of the basement membrane, and edema and angiectasis of lamina propria) were similar in the different groups of patients. We conclude that the inflammatory events leading to the development of the late-phase asthmatic response to allergen represent a stimulus for an increase in the number of mast cells in the bronchial mucosa.     

Nodular regenerative hyperplasia of the liver associated with azathioprine therapy]

Three cases of nodular regenerative hyperplasia of the liver associated with azathioprine therapy are reported. The indication of azathioprine differed in each of the 3 cases including kidney transplantation, graft-versus-host disease following bone marrow transplantation, and suspicion of bowel inflammatory disease, respectively. In all three patients, nodular regenerative hyperplasia was discovered after a prolonged administration of azathioprine (24 to 40 months), with a cumulative dose of 52 to 120 g. Under light microscopy, vascular lesions were associated with nodular regenerative hyperplasia in the 3 cases and consisted of sinusoidal dilatation (2 cases), perisinusoidal fibrosis (2 cases), and peliosis (1 case). In two patients, nodular regenerative hyperplasia was responsible for severe portal hypertension which was treated by portacaval shunt. These findings are strongly suggestive of a role of azathioprine in the occurrence of nodular regenerative hyperplasia. The mechanism of azathioprine-induced nodular regenerative hyperplasia could be related to sinusoidal lesions caused by azathioprine and responsible for liver hypoperfusion, with regenerative hyperplasia in the areas remaining normally perfused. Patients receiving long-term therapy with azathioprine should be followed-up regularly and liver biopsy should be performed when clinical or biochemical liver abnormalities are observed.     

Nodular lymphoid hyperplasia in the gastrointestinal tract in adult patients: A review

Nodular lymphoid hyperplasia of the gastrointestinal tract is characterized by the presence of multiple small nodules, normally between between 2 and 10 mm in diameter, distributed along the small intestine(more often), stomach, large intestine, or rectum. The patho-genesis is largely unknown. It can occur in all age groups, but primarily in children and can affect adults with or without immunodeficiency. Some patients have an associated disease, namely, common variable immu-nodeficiency, selective IgA deficiency, Giardia infection, or, more rarely, human immunodeficiency virus infec-tion, celiac disease, or Helicobacter pylori infection. Nodular lymphoid hyperplasia generally presents as an asymptomatic disease, but it may cause gastrointes-tinal symptoms like abdominal pain, chronic diarrhea, bleeding or intestinal obstruction. A diagnosis is made at endoscopy or contrast barium studies and should be confirmed by histology. Its histological characteristics include markedly hyperplasic, mitotically active germi-nal centers and well-defined lymphocyte mantles found in the lamina propria and/or in the superficial submu-cosa, distributed in a diffuse or focal form. Treatment is directed towards associated conditions because the disorder itself generally requires no intervention. Nodu-lar lymphoid hyperplasia is a risk factor for both intes-tinal and, very rarely, extraintestinal lymphoma. Someauthors recommend surveillance, however, the duration and intervals are undefined.     

Nodular regenerative hyperplasia of the liver.

Nodular regenerative hyperplasia of the liver, an uncommonly reported and poorly defined clinicopathological entity, obscured clinical diagnosis and was misdiagnosed on hepatic biopsy in a recent case. Approximately 19 cases are recorded in the English literature. Six patients had Felty's syndrome, about 12 patients had congestive heart failure, and the patient under discussion had subacute bacterial endocarditis. Light- and electron-microscopic examination was utilized to define nodular regenerative hyperplasia pathologically. Features common to all reported cases are discussed but elucidation of the pathogenesis of nodular regenerative hyperplasia must await further investigation.     

Increased colonic mucosal mast cells associated with severe watery diarrhea and microscopic colitis

Summary A patient with microscopic colitis, clearly identifiable by its histologic characteristics, is presented in whom the histology also revealed large numbers of mast cells. The patient was treated with H₁ antagonists with prompt resolution of the diarrhea. The histologic and therapeutic findings in this case suggest the diarrhea and inflammation of microscopic colitis may be mediated by the degranulation of excessive numbers of mast cells in the colon and small bowel.