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At least 613 records · Page 34Linked to original sources
PubMed1973

Histopathological and electron microscopic studies of lymphangiectasia of the small intestine in Behçet's disease.

The gastrointestinal involvement and immunological findings in 16 patients with Behçet's disease are described. Four of 15 biopsy specimens of jejunal mucosa showed marked lymphangiectasia in the lamina propria similar to the appearance which was thought to be a characteristic finding in protein-losing enteropathy. None of the patients had hypoproteinaemia. Increases in serum immunoglobulin IgA were proved in six of 15 cases; in IgM, five of 15; and in IgG, one of 15. Electron microscopic studies showed that there were thousands of precipitated lymph protein bodies in the extracellular spaces of the lamina propria. Ulcers and healed ulcers of the large intestine were studied by light microscopy.

Adult↗
PubMed1974

The relative importance of the factors involved in the absorption of vitamin E in children.

The vitamin E status and ease of repletion in groups of children with coeliac disease, intestinal lymphangiectasia, and abetalipoproteinaemia was studied and compared with earlier studies in cystic fibrosis and obstructive jaundice. Each group represents an experimental model in which one of the transport steps involved in the absorption of vitamin E is defective or absent and thus the relative importance of these factors could be determined. Chylomicron formation and an adequate intraluminal concentration of bile salts were found to be the most important factors for the efficient absorption of the vitamin. The results in the five groups of patients have therapeutic implications if it is considered desirable to correct vitamin E deficiency states.

Abetalipoproteinemia↗
PubMed1985

Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome.

In 1964 Samman and White described 13 patients with lymphoedema of the lower extremities associated with an unusual dystrophy of the finger and toe nails: this they termed the 'yellow-nail' syndrome. Affected nails were thickened, excessively curved along both axes, very slow growing and of yellowish-grey hue; cuticle and lunula were usually absent and onycholysis was frequently evident. Lower limb lymphangiography in most individuals revealed hypoplasia, or aplasia of the lymphatics, similar to that occurring in primary lymphoedema: other patients also developed pleural effusions of high protein content or ascites suggestive of a more generalised disorder of the lymphatic system. Here we describe a patient in whom the classical 'yellow-nail' syndrome was associated with intestinal and chylous ascites.

Chylous Ascites↗
PubMed1992

Intestinal lymphangiectasia associated with angiofollicular lymph node hyperplasia (Castleman's disease).

A patient presenting with predominantly gastrointestinal symptoms and a history of myocardial infarction was found to have ascites, hepatosplenomegaly, para-aortic lymphadenopathy, thrombocytosis, and a paraproteinaemia. A jejunal biopsy specimen showed lymphangiectasia and histology of the spleen and lymph nodes showed angiofollicular hyperplasia or Castleman's disease of the hyaline vascular type. This association has not previously been described and, moreover, systemic symptoms are unusual in this variant of Castleman's disease.

Castleman Disease↗
PubMed2000

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1983

Macroglobulinaemia and intestinal lymphangiectasia: a rare association.

Two cases in which macroglobulinaemia was associated with intestinal lymphangiectasia are recorded. Immunoperoxidase stains demonstrated a high content of monoclonal IgM in the intestinal lymph. The seven previously recorded examples of this association are reviewed. It is concluded that the concurrence of these two conditions is not merely fortuitous, and that increased viscosity of the lymph consequent on its high IgM content may be important in the pathogenesis of the intestinal lymphangiectasia.

Aged↗
PubMed1993

Waldenström's macroglobulinaemia secreting a paraprotein with lupus anticoagulant activity: possible association with gastrointestinal tract disease and malabsorption.

A 51 year old man with Waldenström's macroglobulinaemia presented with a malabsorptive syndrome related to extensive small bowel lymphangiectasia caused by immunoglobulin accumulation. The patient's plasma had strong lupus anticoagulant activity and the IgM lambda paraprotein displayed specificity for the negatively charged phospholipids phosphatidyl serine and phosphatidyl inositol, as well as the neutral phosphatidic acid. Despite treatment for the macroglobulinaemia the patient died and at necropsy was found to have myocardial ischaemia and segmental infarcts in the spleen and kidney. The coexistence of these relatively rare findings suggests a possible association between Waldenström's macroglobulinaemia with gastrointestinal manifestations and paraprotein specificity for phospholipid.

Antibody Specificity↗
PubMed1989

Vulval lymphangiectasia.

Three cases of lymphangiectasia of the vulva are reported. One case followed Wertheim's hysterectomy for carcinoma of the cervix; two other cases had Crohn's disease with perineal involvement. Lymphangiectasia is a secondary phenomenon resulting from obstruction of previously normal lymphatics. This is in contrast with lymphangioma which is an anatomical abnormality. A review of the literature suggests that the vulval skin may be particularly susceptible to the formation of lymphangiectasia, and our patients' experiences suggest that they are easily mis-diagnosed.

Adult↗
PubMed1975

The lungs in lymphangiomyomatosis and in tuberous sclerosis.

Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases.

Adolescent↗