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)

The clinical and pathological features of 18 new patients with alpha heavy chain disease seen at two referral centres in Baghdad, Iraq, are described. The series included 14 males and four females ranging in age from 14 to 47 years. Almost all patients presented because of long-standing abdominal pain and diarrhoea. The tissue diagnosis and extent of the disease were established at laparotomy in most patients. Peroral jejunal biospy was used in a number of patients, mainly for follow-up. The serological abnormality was confirmed by immunoselection technique. Most of the patients had extensive thickening of the bowel wall and/or tumour masses of the small intestine and mesenteric nodes. Histopathological sections showed muscularis. Preliminary results of the treatment, including two long remissions, are reported. In general, our observations agree with those made by other authors, mostly from the Middle East and Africa. We believe that a high index of clinical suspicion, routine use of the immunoselection, and recognition of the early pathological changes may hopefully lead to the detection of more cases before the frank neoplastic phase of the disease.
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PMID:Alpha heavy chain disease (report of 18 cases from Iraq). 9 95

The histopathology of 145 malignant lymphomas of the small intestine in Iraq have been studied and results compared with the clinical and immunological findings. The most common pathology was an intense mucosal lymphoplasmacytic proliferation effacing the villi and crypts partially or completely. This was either 'pure', usually of mature plasma cells limited to the lamina propria or associated with a fullblown lymphoplasmacytic lymphoma, almost always of the upper small intestine. The syndrome presented as abdominal pain, chronic diarrhoea, clubbing and, sometimes, the serological demonstration of alpha heavy chains. Other types of lymphomas were associated with 'non-specific' mucosal inflammation or follicular lymphoid hyperplasia. They were either lymphocytic, plasmacytic or lymphoblastic with 'starry sky' histiocytic reaction, representing distinct clinicopathological entities unrelated to 'alpha heavy chain disease'. Hodgkin's disease was extremely rare in this series.
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PMID:Primary lymphomas of the small intestine in Iraq: a pathological study of 145 cases. 11 Jun 62

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

Primary small intestinal lymphoma (PSIL) represents a heterogenous group of disorders with variable clinical and pathologic features and a characteristic age, socioeconomic, and geographic distribution. In developed countries, PSIL usually occurs as a localized ileal tumor, shows a bimodal age distribution, and most frequently presents with abdominal pain and obstructive symptoms. Histologically, most of these tumors are diffuse histiocytic, lymphocytic, or undifferentiated lymphomas. Other variants of PSIL, collectively referred to as immunoproliferative small intestinal disease, occur most often among young patients of poor socioeconomic status in Third World countries, mostly in the Middle East and Mediterranean area. They are characterized by involvement of long loops of the upper small intestine and commonly present with abdominal pain, diarrhea, malabsorption, and clubbing of the fingers. A subgroup of these patients shows a serological abnormality with the appearance of part of the alpha heavy chain of IgA in the serum. Histologically, the lesion appears as a dense diffuse lymphoplasmacytic infiltrate of the mucosa of the upper jejenum or duodenum. A form of malignant lymphoma of true histiocytic origin complicates long-standing celiac disease. The contrasting clinical, epidemiological, histopathological, and immunological features of these variants of PSIL raise interesting questions about the pathogenesis of small bowel lymphoma.
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PMID:Primary lymphomas of the small intestine: east-west contrast. 395 68

We report a case of Mediterranean lymphoma treated with antibiotics. A 74-year-old woman visited the hospital due to abdominal pain. Endoscopic examination showed erosions and ulcerations on duodenal mucosa. Biopsy specimens histologically revealed massive infiltration of small-sized lymphocytes and plasma cells in subepithelial mucosa. Immunoperoxidase staining showed that the infiltrating cells were positively stained with anti-alpha heavy chain. Serum IgA concentration was elevated and immunoelectrophoresis of the serum demonstrated monoclonal protein composed of alpha heavy chain. During the antibiotic treatment her symptoms disappeared and serum IgA concentration was normalized. Endoscopic examination also showed healing of the duodenal ulceration. The similarities between Mediterranean lymphoma and gastric mucosa-associated lymphoid tissue (MALT) type lymphoma, both of which may be related to bacterial infection and can be treated with antibiotics, are discussed in this report.
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PMID:"Mediterranean lymphoma" treated with antibiotics. 903 Sep 84

Immunoproliferative small intestinal disease (IPSID) was recently added to the growing list of infectious pathogen-associated human lymphomas. Molecular and immunohistochemical studies demonstrated an association with Campylobacter jejuni. IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain. Geographically, IPSID is most prevalent in the Middle East and Africa. IPSID lymphomas reveal excessive plasma cell differentiation and produce truncated alpha heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the variable heavy chain (V(H)) and the constant heavy chain 1 (C(H)1) sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of C(H)1 domain. Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t(9;14) translocation involving the PAX5 gene. Early-stage IPSID responds to antibiotics (30%-70% complete remission). Most untreated IPSID patients progress to lymphoplasmacytic and immunoblastic lymphoma invading the intestinal wall and mesenteric lymph nodes, and may metastasize to a distant organ. IPSID lymphoma shares clinical, morphologic, and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma, and plasma cell neoplasms.
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PMID:Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms. 1554 84