Regression of mesenteric lymph node cavitation syndrome complicating celiac disease after a gluten free diet

We report the case of a 39-year old woman with celiac disease in association with a cavitating mesenteric lymph node, hyposplenism and intra-hepatic haematopoiesis. The serious initial clinical picture evoked a diagnosis of non-Hodgkin lymphoma but was not confirmed on multiple biopsies. Despite the usual poor prognostic clinical outcome in such a setting, treatment with a strict gluten-free diet resulted in a remarkable persistent improvement in clinical status and lead to almost complete regression in radiological signs observed for up to 30 months follow-up.     

Multifocal small bowel lymphoma and latent celiac sprue

Malignant small intestinal lymphoma may complicate or antedate clinical recognition of celiac sprue, a disorder becoming increasingly diagnosed as a subclinical or occult disease. A 73-yr-old woman with previously resected jejunoileal lymphoma and normal proximal small bowel biopsy specimens was given a high-gluten diet containing 40 g of added gluten daily for 4 wk. This caused small intestinal biopsy abnormalities typical of celiac sprue; the abnormalities resolved 6 wk later with a gluten-free diet. This indicates that latent celiac sprue may be present in some patients with lymphoma and suggests that the association of celiac sprue and lymphoma may be more frequent than is currently appreciated.     

Mesenteric lymph node cavitation syndrome

The mesenteric lymph node cavitation syndrome consists of central necrosis of mesenteric lymph nodes and may occur with either celiac disease or a sprue-like intestinal disease that fails to respond to a gluten-free diet. Splenic hypofunction may also be present. The cause is not known but its development during the clinical course of celiac disease is usually indicative of a poor prognosis for the intestinal disorder, a potential for signif icant compli-cations including sepsis and malignancy, particularly...     

Cavitating mesenteric lymph node syndrome: a rare complication of celiac disease

Among the many complications of celiac disease, mesenteric lymph node syndrome cavitated is considered one of the rarest, there is few case series published in the literature. The etiology and pathophysiology are unknown but because of its high mortality rate, estimated to be around 50%, it should recognize at an early stage in order to institute appropriate therapy as soon as possible.     

A unique combination of celiac disease, mesenteric lymph node cavitation, splenic atrophy and necrotizing hepatitis

We report on a patient who was diagnosed six years before with celiac disease, with a current combined problem of asplenism, mesenteric cysts and elevated liver function tests. The implications of splenic atrophy mimic those of post-splenectomy patients. Mesenteric lymph node cavitation is a rare complication of celiac disease that is most often associated with splenic atrophy. The pathogenesis is unknown. The clinical implications of the cavitated mesenteric lymph nodes are unclear. The association of celiac disease with liver disease was reported many years ago, but only recently these associations have been more clearly defined. Liver involvement shows a clinical spectrum varying from nonspecific reactive hepatitis, chronic active hepatitis, steatohepatitis to frank cirrhosis. Associations with autoimmune hepatitis, autoimmune cholangitis, primary biliary cirrhosis and primary sclerosing cholangitis have been described. In our patient, we found no obvious cause for the necrotizing hepatitis and the negative auto-antibodies made it impossible to firmly establish the diagnosis of autoimmune hepatitis. The causal relationship with celiac disease, if any, remains unproven.     

Mesenteric lymph node cavitation: a rare hallmark of celiac disease

The cavitation of mesenteric lymph nodes represents a rare complication of celiac disease (only 30 reported cases) whose pathogenesis remains to be clarified. We here report the case of a 67-year-old woman referred to us because of a malabsorption syndrome lasting for 2 years; massive lymph node enlargement and cavitation were detected by means of ultrasonography and a computed tomography scan. Celiac disease was definitely diagnosed by means of duodenal histology, and a laparotomy was performed to exclude an underlying T-cell lymphoma. The adoption of a gluten-free diet led to a rapid and dramatic improvement in the clinical and histologic picture and normalization of the size of the lymph nodes. Celiac disease should be considered in the differential diagnosis of all patients with mesenteric lymph node cavitation.     

Primary abdominal lymphoma. Presenting manifestation of celiac sprue or complicating dermatitis herpetiformis.

In patients with celiac sprue the risk of having a primary abdominal lymphoma is increased. We have studied the lymphoma aspect of this celiac sprue-lymphoma relationship. During a four year period, seven patients with abdominal lymphoma were found on subsequent investigation to have celiac sprue or the closely linked disorder, dermatitis herpetiformis. Five of the patients had a small intestinal lymphoma, one had a gastric lymphoma and one had a retroperitoneal lymphoma. Histologically, six of the seven tumors were of a diffuse histiocytic type and one was undifferentiated. In four patients the ulcerating small intestinal lymphoma was initially misinterpreted as “benign ulcerative nongranulomatous jejunoileitis,” a condition also reported to complicate celiac sprue. In several patients the associated celiac sprue was clinically occult and could readily have been missed. The celiac sprue was fully responsive in five of the patients who were treated with a gluten-free diet. Our studies suggest that celiac sprue and abdominal lymphoma occur together more often than is currently appreciated. The frequency of this association may be overlooked for two reasons: the associated celiac sprue is sometimes mild and may remain undetected; and, early ulcerative intestinal lymphoma may be mistaken for “benign ulcerative nongranulomatous jejunoileitis.” Patients with abdominal lymphoma should be investigated for celiac sprue and for dermatitis herpetiformis because of the nutritional and pathogenetic implications.     

On the pathogenesis and clinical course of mesenteric lymph node cavitation and hyposplenism in coeliac disease

BACKGROUND: Coeliac disease is a disorder characterised by malabsorption related to abnormal small bowel structure and intolerance to gluten. There are several reports of an increased risk for malignancy in coeliac disease and its relation to gluten-free, reduced gluten, or normal diet. While a normal diet is associated with an excess of cancer of the mouth, pharynx, oesophagus, and also of lymphoma, treatment with a gluten-free diet restores the cancer risk back to normal. PATIENT: In the present study, we report on a 63-year-old female patient with a history of coeliac disease for twenty years who presented with persistent diarrhoea, weight loss, and an abdominal mass. RESULTS: The gastroenterological work-up revealed small bowel mucosal atrophy, absence of functional splenic tissue, and evidence for an involution of a mesenteric lymph node, termed cavitation. DISCUSSION: This triad has been previously described to represent a rare disease entity related to coeliac disease. We report a two-year follow-up and a review of the literature on the pathogenesis, prognosis, and therapeutical implications of this disease entity.     

Testing for celiac sprue in irritable bowel syndrome with predominant diarrhea: a cost-effectiveness analysis

BACKGROUND & AIMS: Some patients with diarrhea-predominant irritable bowel syndrome (IBS-D) may have undiagnosed celiac sprue (CS). Because the symptoms of CS respond to a gluten-free diet, testing for CS in IBS may prevent years of morbidity and attendant expense. We sought to determine whether this might be a cost-effective diagnostic strategy in IBS-D. METHODS: We used decision analysis to calculate the cost-effectiveness of 2 competing strategies in IBS-D: (1) start empirical IBS treatment and (2) perform serologic test for CS followed by endoscopic biopsy for positive tests. The base-case cohort had a CS prevalence of 3.4%, which was varied between 0% and 100% in sensitivity analysis. The outcome measure was cost per symptomatic improvement. RESULTS: Under base-case conditions, testing for CS instead of starting empiric IBS therapy cost an incremental $11,000 to achieve one additional symptomatic improvement. Testing for CS became the dominant strategy when the prevalence of CS exceeded 8%, the specificity of CS testing exceeded 98%, or the cost of IBS therapy exceeded $130/month. The incremental cost-effectiveness of testing for CS exceeded $50,000 when the prevalence fell below 1%. CONCLUSIONS: Testing for CS in patients with IBS-D has an acceptable cost when the prevalence of CS is above 1% and is the dominant strategy when the prevalence exceeds 8%. The decision to test should be based on a consideration of the population prevalence of underlying CS, the operating characteristics of the screening test employed, and the cost of proposed therapy for IBS.     

Calcified cavitating mesenteric lymph node syndrome: Case presentation and literature review

Cavitating mesenteric lymph node syndrome (CMLNS) is a rare complication of celiac disease. Globally, only 36 cases of CMLNS have been reported to date. The present article reports an incidence of its unique pathology and possible complications of celiac disease, followed by a review of the syndrome. A case involving a 51-year-old man with celiac disease who was referred to hospital because of a non-tender abdominal mass is described. Plain film x-ray of the abdomen revealed fine curvilinear calcifications in the left lower quadrant. A complex, cystic-appearing, lobulated mass with somewhat echogenic walls most consistent with calcifications was revealed on subsequent ultrasound (US) imaging. Colour Doppler imaging showed no evidence of vascularity within the lesion. Computed tomography (CT) imaging showed a thin rim of calcification in the walls of multiple cystic components. Enhanced magnetic resonance (MR) imaging revealed a mixed solid and cystic multiloculated mass, with fat-fluid layers originating from the root of the small bowel mesentery. A CT-guided biopsy was performed. The fine-needle aspirate revealed calcified matter with no associated cellular material. No malignant cells were seen; CMLNS was established as the diagnosis. To the authors’ knowledge, there are no previous reports in the literature describing the finding of rim calcification on US or MR imaging in the setting of CMLNS. CMLNS is an important diagnosis to consider, particularly in patients with a history of celiac disease. The finding of rim calcification on US in the setting of cavitating mesenteric adenopathy should prompt further diagnostic imaging studies such as CT or MR imaging. These may lead to additional pathology studies such as a CT-guided biopsy to further characterize the lesion at the cellular level, to investigate potential malignancy and to further guide follow-up and patient management.     

Intestinal lymphoma and mesenteric panniculitis: complications of undiagnosed celiac disease

Celiac disease is the most common severe food intolerance in the Western world and is due to gluten ingestion in genetically susceptible children and adults. The key treatment in these patients is a gluten-free diet, because most complications are more common when dietary compliance is poor. The most serious complication of celiac disease is the development of neoplasms (the most common of which is enteropathy-associated T-cell lymphoma). However, a number of reports have indicated an increased prevalence of ulcerative jejunitis and extraintestinal manifestations, including chronic hepatitis, fibrosing lung disease, and epilepsy syndromes. We report the case of a 53-year-old-man with long-standing diarrhea; because celiac disease was not suspected, the patient developed celiac-associated T-cell lymphoma and mesenteric panniculitis.     

恶性黑色素瘤小肠转移、系膜淋巴结转移及脑转移1例

恶性黑色素瘤转移至胃肠道常见的部位为小肠结直肠肛门,同时转移至小肠、系膜淋巴结及颅内者未见文献报道.本例恶性黑色素瘤伴有系膜淋巴结转移及脑转移,没有典型的临床表现,术中所见颅内肿物与小肠及系膜淋巴结肿物颜色质地不一致,术后病理对确定腹部及颅内肿物来源至关重要,患者病史对鉴别原发或转移性黑色素瘤意义重大.     

Steatorrhea and malignant lymphoma the relationship of malignant tumors of lymphoid tissue and celiac disease

Summary 1. Seven patients with celiac disease complicated by malignant lymphoma are presented. Seventeen other cases of steatorrhea and lymphoma, together with 40 cases from the literature, are discussed.2. The commonest mode of presentation of this type of reticulosis is failure of response to treatment for steatorrhea or a recurrence of symptoms in patients who have improved as a result of treatment. Abdominal pain, skin rashes, fever, and an abdominal mass occur frequently. Laparotomy with biopsy gives the best chance of diagnosis.3. When steatorrhea occurs with malignant lymphoma, the tumor is found mostly commonly in proximal small intestine; the incidence of Hodgkin's disease is increased; there appears to be a higher than normal incidence among women; the onset of lymphoma is later than usual, and the prognosis is poorer.The authors wish to thank the many clinicians who allowed us to study cases under their care and the following pathologists for help in studying histological material: Dr. N. J. Brown, Dr. J. A. Dossett, Dr. R. A. Drury, Prof. C. V. Harrison, Dr. R. J. Sandry, and Dr. T. H. Shaw.