Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten cases of primary upper small-intestinal lymphoma associated with alpha-chain protein in serum were discovered in a prospective study of the sera of patients with immunoproliferative small-intestinal disease (IPSID). Patients were mostly young males presenting with abdominal pain, weight loss, and diarrhea and showing laboratory evidence of carbohydrate, fat, and vitamin B12 malabsorption and hypoalbuminemia. The more frequently encountered pathologic abnormality was a diffusely nodular jejunal mucosa produced by a plasmacytic infiltrate of variable cell maturity involving a varible depth of small bowel wall with or without involvement of the mesenteric or para-aortic-lymph node complex and, in one instance, the liver. A less frequent picture included circumferential ulcerative and constrictive transmural tumors of the upper small intestine produced by a malignant lymphoma with involvement of abdominal lymph nodes. Small-intestinal surface epithelial abnormalities, a dense mantle of mature plasma cells overlying the lymphoma, a pronounced follicular lymphoid hyperplasia adjacent to and at distances from the lymphoma were other features of note in our IPSID cases associated with alpha-chain protein.
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PMID:Primary upper small-intestinal lymphoma and alpha-chain disease. Report of 10 cases emphasizing pathological aspects. 41 96

The clinical, biochemical, immunological and histopathological features in a patient with alpha-chain disease are described. The patient, a 20-year-old Coloured man, presented with severe steatorrhoea, malabsorption, abdominal pain and progressive general deterioration. An heterogeneous abnormal band with IgA immunochemical specificity was detected on electrophoresis of the patient's serum and urine. This protein was identified as free alpha-chain and was present in serum, urine, saliva and jejunal juice. A jejunal mucosal biopsy specimen showed distinctive appearances associated with alpha-chain disease. Bone marrow involvement was found and abnormal lymphoid cells were seen in the circulation together with an increased B lymphocyte population derived from bone marrow. This is the third South African patient with alpha-chain disease to be diagnosed. The patient has shown a partial remission after 12 months' chemotherapy. There was rapid symptomatic response and normalisation of protein parameters which were not paralleled by an objectively discernible response as assessed by haematological examination, intestinal absorption studies and histology of the jejunal mucosa.
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PMID:Alpha-chain disease in a non-Mediterranean climate. A case report. 81 7

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

We describe a female aborigine from Taiwan with alpha-chain disease associated with multiple polypoid intestinal lymphocytic lymphoma and leukemic manifestation. Initially, the patient experienced intermittent diarrhea, abdominal pain, and leukemic manifestation. No evidence of bone marrow involvement was found. Alpha-chain protein was demonstrated in the serum. Gastroendoscopy and a series of radiographs of the small intestine revealed multiple polypoid tumors involving the entire length of the small intestine. Duodenal biopsy showed diffuse lymphocytic lymphoma. Immunohistochemical staining of tumor samples revealed features typical of alpha-chain disease. Cytogenetic analysis showed the same abnormal karyotypes of neoplastic clones in intestinal tumor cells and in circulating leukemic cells. The data suggest that alpha-chain disease can present initially with intestinal multiple polypoid lymphocytic lymphoma and leukemic manifestation without evidence of bone marrow involvement. The data also support the homing theory of lymphomas from mucosa-associated lymphoid tissue.
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PMID:Leukemic manifestation in a case of alpha-chain disease with multiple polypoid intestinal lymphocytic lymphoma. 141 95

Clinical, immunological, and histological recovery in a patient with alpha-chain disease is described. The patient, a 27-year-old Greek man, presented with severe steatorrhoea, abdominal pain, oedema, and hypogammaglobulinaemia. Treatment with tetracycline produced only temporary remission. Intermittent therapy with prednisone and cyclophosphamide together with antibiotics was followed by clinical recovery, return of histological appearances of the small intestine to normal, and disappearance of free alpha-chain protein from the serum. The patient remained well one year later without treatment.
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PMID:Alpha-chain disease with clinical, immunological, and histological recovery. 413 71