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)

Primary lymphomas of the small intestine (PIL) show definite racial and geographic distribution. Clinical and laboratory data on 132 patients with PIL treated and followed up over a 14-year period in Baghdad, Iraq, are presented. Based on the pattern of involvement of the bowel and the histopathologic appearance of the tumor, the patients can be divided into three main groups. Ninety-seven patients had diffuse lymphoplasmacytic infiltration affecting large segments of the upper bowel. Abdominal pain, anorexia, weight loss, diarrhea, malabsorption, and clubbing of the fingers are the most common clinical findings. The barium appearance of the small intestine and peroral jejunal biopsy specimens are abnormal in nearly all cases. This clinicopathologic entity has been referred to in the literature as Mediterranean lymphoma (ML). Ten of 34 patients tested had free alpha-heavy chain in the serum. Twenty-three cases had other "Western" variants of lymphoma (18 lymphocytic and 5 plasmacytic). The lesions were localized, occurring most frequently in the lower ileum or ileocecal area. The most common presentation was intestinal obstruction. Twelve children had Burkitt's lymphoma (BL), presenting most commonly with abdominal masses and/or intestinal obstruction. These patients responded poorly to cyclophosphamide, and the disease disseminated early and extensively. None of the patients with the localized lymphomas or BL had free alpha chain in the serum.
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PMID:Clinical and pathologic subtypes of primary intestinal lymphoma. Experience with 132 patients over a 14-year period. 641 50

Gastrointestinal lymphoma, uncommon in the West, is far more prevalent in developing countries where it falls into two groups: 'Western'-type lymphomas, similar to those seen in developed countries, and the so-called Mediterranean-type lymphoma. It is now accepted that Mediterranean lymphoma represents, in the majority if not in all cases, the late stage of alpha heavy chain disease (alpha-HCD). This disease is characterized by abnormal secretion of an immunoglobulin fragment; alpha-HCD and Mediterranean lymphoma constitute two ends of a spectrum of pathology now classified as immunoproliferative small intestinal disease (IPSID). IPSID is associated predominantly with poor socioeconomic conditions; patients present with progressive malabsorption in the second and third decades of life. Diagnosis is established by small bowel biopsy, with or without high serum levels of the alpha heavy chain protein. Treatment consists of an initial staging laparotomy, with debulking of lymphomatous deposits if appropriate, followed by chemotherapy or radiotherapy. Overall prognosis is poor but the recent use of doxorubicin-based chemotherapy offers some hope for the future.
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PMID:Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease. 831 11

Immunoproliferative small intestinal disease (IPSID) is prevalent in the Mediterranean region and in many Third World countries but is rare in Southeast Asia. Between 1980-1990, 4 cases of IPSID were admitted to Ramathibodi Hospital, Bangkok. Three were males and the mean age was 32 +/- 20.2 years. All patients presented with chronic diarrhea of 7 months to 6 years duration, and weight loss of 15 to 31 kg. All were malnourished, three cachectic, and one patient showed growth retardation. Intestinal parasites were found in all cases: two had multiple infections and three had uncommon protozoal infections (coccidium, cryptosporidium). Barium radiographs revealed intestinal mucosal fold thickening with malabsorption pattern in all cases. Alpha chain IgA was detected in one patient. The remainder underwent exploratory laparotomy and the histological finding was of plasma lymphocytic infiltration of the small intestinal mucosa. All patients responded to oral tetracycline with complete remission occurring in one case. During the follow-up period, 3 cases had progressive retractable clinical courses but all died 2 to 5 years after the diagnosis. The causes of death in these patients were secondary bacterial infection (1 case), intestinal tuberculosis (1 case), fungal infection (1 case) and immunoblastic sarcoma in another case. The results of this study confirm the occurrence of IPSID in Thailand. IPSID responds to oral antibiotic therapy and complete remission may be achieved during the early reversible benign phase, thus an awareness of its occurrence is of clinical importance.
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PMID:Immunoproliferative small intestinal disease (IPSID) in Thailand. 836 83

Immunoproliferative small intestinal disease (IPSID) is a poorly recognized cause of malabsorption syndrome in India. Clinicopathological features of five patients with IPSID seen over a two-year period are described. Our data suggest that IPSID is commonly misdiagnosed as intestinal tuberculosis due to lack of awareness and reluctance to obtain small bowel biopsies. Empirical institution of anti-tubercular chemotherapy not only leads to delayed diagnosis but also possibly alters the natural history of the disease, resulting in an intermediate phase of amelioration followed by a terminal phase of lymphomatous transformation. The disease is therefore usually diagnosed at an advanced stage and hence is associated with a relatively poor outcome.
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PMID:Immunoproliferative small intestinal disease: a frequently missed diagnosis. 916 88

Immunoproliferative small intestinal disease (IPSID), a proliferative disorder affecting the intestinal immune system, has only been reported sporadically in India. Fifteen patients with malabsorption syndrome who were diagnosed to have IPSID were included in this study. Mucosal biopsies from all patients, full thickness surgical biopsies from 10 and autopsy material from four patients were examined by light microscopy and immunohistochemistry. The patients were predominantly young (aged 16-36 years) and male (13 of 15). Diarrhoea, weight loss, vomiting and abdominal pain were the major symptoms. The upper small bowel was involved in all cases. Involvement of large bowel was detected antemortem in three patients, but was found in all autopsied patients. Involvement of the stomach was noted in one patient at autopsy. Mesenteric lymph nodes were involved in all patients who underwent laparotomy. The plasmacytic infiltrate was uniformly positive for alpha-heavy chain, and either negative for light chain production or showed monotypic light chain production. Some of the blasts were also positive for alpha-heavy chain. Three patients died before therapy could be commenced. One patient with stage A disease is alive and clinically free of disease at 7 years. Of the remainder, there have been four long-term survivors with chemotherapy. Immunoproliferative small intestinal disease occurs in southern India and has characteristics similar to that in other parts of the world. Early diagnosis may improve outcome in this disease.
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PMID:Immunoproliferative small intestinal disease in South India: a clinical and immunomorphological study. 991 27

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

Immunoproliferative small intestinal disease is a distinctive lymphoproliferative disorder. Among these disorders, it is the only disease associated with a specific and characteristic abnormal protein, and also an identifiable, at least in some patients, early phase with a benign-looking histo-pathologic expression. Whether the disease at this stage is malignant or not is not known. Treatment of this early phase with antibiotics may cause remission in some patients. This observation is significant and raises the question of chemoprevention in lymphomas. In contrast to primary nonimmunoproliferative small intestinal lymphomas, in which the pathology in the intestine is usually focal and involving specific segments of the intestine and leaving the segments between the involved areas free of disease, the pathology in immunoproliferative small intestinal disease is diffuse, with a mucosal cellular infiltrate involving large segments of the intestine and sometimes the entire length of the intestine, thus producing malabsorption. Preliminary recent epidemiological data have shown a decrease in the incidence of this disease in endemic areas, and therefore environmental factors are suspected to play a major role in its pathogenesis. Additional research is indicated not only to understand this specific lymphoproliferative disorder but also to understand lymphomas in general.
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PMID:Immunoproliferative small intestinal disease: current concepts. 1625 67

Alpha heavy chain disease is a rare affection in the West and reported mainly in developing countries with the improvement of hygienic conditions, the disease become rare in Tunisia, the last case was reported in 1991. The aim of the study is to report a new Tunisian case and to describe clinical, endoscopical and histological characteristics of the disease. The patient in a 37 years old male, presented with chronic diarrhoea associated with malabsorption syndrome. The diagnosis of alpha heavy chain disease was confirmed by histological examination of the resected intestine after surgery for intestinal obstruction. The patient received chemotherapy. The follow up is of two months.
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PMID:[Alpha heavy chain disease. A Tunisian case]. 1726 8


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