Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and histopathologic features in seven patients with intestinal lymphoma are reported. Three of these presented with ulcerative jejunitis and four with overt lymphomas. A short history of abdominal pain with weight loss followed by intestinal obstruction, hemorrhage, or perforation characterized all the patients except one in whom a nine year history of malabsorption preceded the acute phase of the disease. Malabsorption was demonstrated in four of the patients, and all showed villous atrophy with crypt hyperplasia of the jejunum remote from areas of ulceration or frank lymphoma. The malignant lymphoma cells showed varying degrees of pleomorphism and exhibited phagocytosis of platelets, red cells, and cell debris. The accompanying infiltrate of inflammatory cells often overshadowed the neoplastic histiocytes, and in those cases showing little pleomorphism these cells could be easily overlooked. In the intestine the tumor cells were usually present as a diffuse infiltrate in the lamina propria or within the bases of ulcers and in five of seven cases did not give rise to macroscopic tumor masses. In all patients dissemination of tumor cells to the lymph nodes, liver, spleen, and bone marrow was evident, the infiltrate in all these organs resembling that seen in malignant histiocytosis. The morphology of the tumor cells, their phagocytic nature, the diffuse character of the tumor infiltrate, and the pattern of dissemination suggest that this lesion should be designated malignant histiocytosis of the intestine rather than histiocytic lymphoma (reticulum cell sarcoma). It is suggested that the tumor may arise from cells of monocyte-histiocyte lineage normally present in the lamina propria of the gut and that a prolonged cryptic phase accompanied, and often overshadowed, by an inflammatory reaction may give rise to malabsorption and ulcerative jejunitis before overt lymphoma is manifest.
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PMID:Malignant histiocytosis of the intestine. Its relationship to malabsorption and ulcerative jejunitis. 73 Jan 48

A 20-year-old Persian man with Middle Eastern lymphoma is described, and 84 additional cases from the literature are reviewed. Basically, the disease is a malignant lymphoma which involves the upper small intestine (duodenum and proximal jejunum). It is associated with clubbing of the fingers, abdominal pain, weight loss, diarrhea, vomiting, and malabsorption, and frequently occurs in a younger age group than "Western Hemisphere" intestinal lymphoma. Some patients also have alpha heavy chain disease. The sex ratio is equal, and the disease occurs only in Middle Eastern and North African Moslems and Jews. Upper gastrointestinal radiographs are frequently diagnostic, and per oral small intestinal biopsy is nearly always diagnostic. Pathologically, the following features are characteristic for Middle Eastern lymphoma: partial or total villous atrophy with only mildly abnormal surface epithelium, sparsity of crypts, lymphatic dilatation, and infiltration of lamina propria by pleomorphic mononuclear cells which pepetrate the muscularis mucosa. The etiology and pathogenesis of this disease are unknown, but several hypotheses are discussed. Treatment by a variety of modalities is far from satisfactory, and the prognosis is much poorer than that observed in patients with the "Western" form of intestinal lymphoma. Other differences between Middle Eastern lymphoma and Western lymphoma are described in detail,
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PMID:Middle Eastern intestinal lymphoma: report of a case and review of the literature. 78 12

Clinical, immunochemical and pathological studies were performed in a 27-year-old male who presented with abdominal pain, the malabsorption syndrome, and marked weight loss. Immunoelectrophoretic studies revealed alpha-heavy chains in the patient's serum, urine and intestinal juice. Treatment with cyclophosphamide and corticosteroids produced an improvement in the patient's condition and he survived for 18 months after the onset of symptoms. Autopsy revealed a malignant lymphoma of mixed histiocytic-lymphocytic type involving mainly the mesenteric lymph nodes, but also affecting the jejunal mucosa, liver, spleen and extraabdominal lymph nodes. This is the first case of abdominal lymphoma with alpha-heavy chain disease to be reported from Yugoslavia.
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PMID:Abdominal lymphoma with alpha-heavy chain disease. 80 83

Primary intestinal lymphoma in young adults is a disease that occurs mainly in underprivileged populations. There is evidence that in some cases this disease evolves from a benign lymphoplasmocytic infiltration of the gut with alpha heavy chain. More studies are needed on the effect of environmental and genetic factors on the evolution of this disease. The role of oncogenic viruses in the development of intestinal lymphoma with malabsorption is an open question. Regional studies on the entity of intestinal lymphoma with malabsorption and its relationship to childhood lymphoma in the same populations are warranted.
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PMID:Primary intestinal lymphoma and its relation to alpha heavy chain disease. 81 Jan 50

Two patients with alpha heavy chain disease are described. In the first patient, treatment with cyclophosphamide, prednisone and doxycycline was associated with a 28 month-long remission and the disappearance of the paraprotein and lymphoplasmocytic infiltration of the intestine. Shortly afterwards, a retroperitoneal immunoblastic lymphoma was found associated with an immunoglobulin G-kappa-paraproteinemia, and gamma heavy and kappa-light chains in the urine; the intestinal biopsy specimen was normal. In the other patient, the alpha chain only appeared two years after the malabsorption syndrome. The fact that in the first, apparently cured patient, a tumor of different anatomic site and secretory capacity appeared, suggests the existence of a B-cell neoplasia of different clone from that which gave rise tothe original disease. In the second patient, it is probable that only the increase in the mass of neoplastic cells led to the detection of the protein abnormality, or alternatively the antigenic-oncogenic stimulus led to the abnormal secretion only after two years.
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PMID:The evolution of alpha heavy chain disease. 81 98

The clinical and pathological features of 17 "Western" type primary abdominal lymphomas (WTL) are compared with 14 of "Mediterranean" type (MTL). The MTL involved only young adult Mulatto and African patients in whom malabsorption and abdominal pain were the major clinical features. The WTL also predominantly affected Mulatto patients but four cases occurred in Caucasians, and the mean age at presentation was two decades later. An obstructive presentation was the most common; only one patient in this group had evidence of malabsorption. The WTLs were located mainly in the distal small bowel and were nearly all of monomorphic lymphocytic or histiocytic type. The MTLs were sited mainly in the duodenum and jejunum and were of an unusual pleomorphic histologic type. A spectrum of cells from those resembling atypical lymphocytes to large histiocytic types were seen, some of the latter resembling Reed-Sternberg cells. The pattern of mesenteric node infiltration in the MTLs was also unusual in that preservation of the medullary sinuses was a common finding. A notable feature of the MTLs was the presence of a heavy infiltration of mature-looking plasma cells associated with a fairly severe villous atrophy in the lamina propria of the small bowel. In the WTLs the adjacent small bowel did not show this feature. In addition to the above cases three patients with a similar heavy plasma cell infiltrate and villous atrophy but without evidence of a lymphoma are described. These cases may represent examples of MTL in a pre-malignant phase. Also included in this study are three patients with alpha-chain disease (alpha-CD), all with a heavy plasma cell infiltration and villous atrophy of the lamina propria and a pleomorphic type lymphoma involving the mesenteric nodes in all, and the small bowel in two. The lymphomas in alpha-CD have been interpreted as immunoblastic sarcoma by Lukes and Collins. Both genetic and environmental factors may be operative in the MTLs including the cases of alpha-CD.
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PMID:Primary intestinal lymphoma of "Western" and "Mediterranean" type, alpha chain disease and massive plasma cell infiltration: a comparative study of 37 cases. 82 14

Twenty-seven intestinal lymphoma patients were studied. Abnormal alpha-chain immunoglobulin was detected in the sera of seven of these patients. The alpha-chain disease patients were from the rural areas of Southern Iran. They were of low socio-economic status and their age ranged from 15-44 years. Predominant clinical features were malabsorption, diarrhoea , abdominal pain, vomiting, and weight loss. Infiltration of mucosa of the small intestine with plasma cells and also distortion and flattening of the villi were common histopathologic characteristics of these patients. Involvement of mesenteric lymph nodes with infiltration of tumour cells was observed in a number of cases. Protein studies revealed no significant differences between the serum immunoglobulin levels of these patients and normal values. Immunoelectrophoresis using monospecific antiserum against H-chain of human IgA demonstrated the abnormal precipitin band of alpha-chain disease protein.
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PMID:Alpha-chain disease and its association with intestinal lymphoma. 82 61

A 30-year-old Turk was admitted with signs of exudative enteropathy together with malabsorption. There was no improvement on a gluten-free diet. Immunological investigations demonstrated atypical IgA-immunoglobulin in the serum which did not precipitate with antisera against L-chains. Peroral ileal biopsy and surgical biopsy material showed a diffuse proliferation of plasma cells in an altered ileal mucosa and in the mesenteric lymph nodes. Skeletal X-rays showed no osteolysis and the bone marrow showed no evidence for multiple myeloma. Treatment with melphalan and steroids resulted in a three year remission. In the terminal stage an intra-abdominal malignant lymphoma developed.
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PMID:[Alpha-chain disease presenting as malabsorption syndrome with exudative enteropathy (author's transl)]. 82 83

Evaluation of stored iron by means of DFOX-induced sideruria in 101 subjects with various degree of hyposideraemia with or without anaemia, is reported. Three groups were examined: 49 patients with chronic loss of blood and malabsorption and urinary iron values up to 1 mg/24hr; 43 with non-bleeding neoplasia, collagen disease, lymphoma, cirrhosis of the liver etc. and values of 1-2mg/24 hr; 9 with rheumatoid arthritis and cirrhosis of the liver and values over 2 mg/24 hr. The reasons why hyposideraemia may accompany incipient of frank tissue hypo-, normo- or hypersiderosis are discussed.
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PMID:[Desferrioxamine in the diagnosis of hypo-, normo-, and hypersiderotic hyposideremia]. 84 86

Malabsorption studies were performed on five Iranian patients with primary small intestinal lymphoma. The effect of oral tetracycline (1.0 g daily) was also studied in three of the above subjects. The results of breath tests (utilizing glycine-1-14C-cholic acid) were abnormal in all five subjects before the antibiotic treatment. Oral tetracycline had a striking effect towards normalizing the results of breath tests. Schilling tests (with intrinsic factor) improve in two patients and steatorrhea improved in all and there was significant weight gain. The antibiotic had no apparent effect on D-xylose or folate absorption tests. It is concluded that bacterial overgrowth in the small intestinal lumen is an important contributory factor to the malabsorption syndrome of this disease.
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PMID:Role of bacterial overgrowth in the malabsorption syndrome of primary small intestinal lymphoma in Iran. 87 55


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