Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphomas can be localized to the gastrointestinal tract and can be primitive or secondary to a systemic lymphoma. Lymphomas can be classified as Hodgkin's and non-Hodgkin's. The latter include IPSID (all of the B-cell) and non-IPSID lymphomas. The involvement of the small intestine in its entire length is a feature of IPSID lymphomas. Non-IPSID lymphomas include MALT-type, which is a B-cell lymphoma, and EATCL, a T-cell lymphoma which is a not infrequent complication of coeliac disease. There are several classifications of lymphomas: the one by Isaacson is the most widely used at present.
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PMID:[Nosography of intestinal lymphomas]. 853 65

Immunoproliferative small intestinal disease (IPSID) is mostly found in young adults of low socioeconomic class in developing countries. This condition is characterized by a dense lymphoplasmacytic infiltrate beneath the epithelium in the duodenal and proximal jejunal mucosa and in the mesenteric lymph nodes. In two thirds of cases, the involved lymphocytes elaborate an anomalous alpha-heavy chain protein. The etiology of this disease is unclear, although various parasitic, genetic, and toxic mechanisms have been proposed. Half of all IPSID patients will be found at diagnosis to have a concurrent intestinal B-cell lymphoma, and most of the remaining patients develop frank lymphoma within a few years. Although most reports of IPSID are from developing nations or indigent immigrant populations within Western countries, four cases of an IPSID-like condition have been documented in white women. Furthermore, although many IPSID patients progress to high grade indeterminate-type lymphoma within a few years of initial presentation, there have been occasional reports of long term survival without lymphomatous conversion. Here, we present an atypical case of IPSID--a California native who, though of Mexican heritage, had resided in the United States his entire life and did not belong to an indigent population. This patient had biopsy-proven IPSID that progressed over 30 yr but never exhibited lymphomatous conversion despite end stage intestinal stasis and recurrent obstruction, culminating in death. Our case calls into question some current assumptions about the prelymphomatous nature of this disease.
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PMID:Immunoproliferative small intestinal disease: prolonged 30-year course without development of lymphoma. 1259 Oct 78

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

This study describes the frequency, demographics, clinical presentation, endoscopic findings, histopathological features, treatment and outcome of 'Immunoproliferative small intestinal disease' (IPSID). Archives contained a total of 27 cases of IPSID diagnosed and treated over an 18-year period. A M: F ratio of 2.4:1 was seen with a mean and median ages of 28.7 and 25 years. Most patients (68.8%) presented with abdominal pain and diarrhoea. In the majority (62.5%), duodenum was the primary site of involvement. Endoscopy showed polypoidal, raised or flat lesions. Biopsy findings included blunting or flattening of villi with dense plasma cell infiltrate and lymphoepithelial lesions. Twenty-four cases were categorized as stage A and B (benign and intermediate) and three were categorized as stage C (malignant, diffuse large B-cell lymphoma with plasmacytoid features). Stage A and B patients responded well to antibiotic treatment (tetracycline) with regression of the lesions while for stage C patients standard CHOP chemotherapy was administered.
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PMID:Immunoproliferative small intestinal disease (IPSID). 2127 91

Immunoproliferative small intestinal disease (IPSID) is an extra-nodal B-cell lymphoma most commonly described in the Mediterranean, Africa, and Asia. It is associated with poverty and poor sanitation, and is rarely encountered in developed countries. A 26-year-old previously healthy, Marshallese male was transferred to our facility with a 6-month history of watery diarrhea, weakness, and cachexia refractory to multiple short courses of oral antibiotics. Stool cultures grew Campylobacter jejuni and Vibrio fluvialis. Endoscopic evaluation showed histologic evidence of Helicobacter pylori gastritis and gross evidence of whipworm infection found in the colon. Mesenteric lymph node biopsy cultures grew Escherichia coli. Histopathology and immunohistochemical stains of the small intestine were consistent with IPSID. He subsequently transformed to diffuse large B-cell lymphoma (DLBCL) with tonsillar involvement despite treatment with rituximab and an extended course of antibiotics. Systemic chemotherapy with six cycles of rituximab, cyclophosphamide, vincristine, doxorubicin, prednisone, and lenalidomide, resulted in remission of his diffuse B cell lymphoma. This case is illustrative of IPSID developing in a previously healthy individual due to overwhelming polymicrobial gastrointestinal infection by C. jejuni and other enteric pathogens with subsequent transformation to an aggressive DLBCL. IPSID should be considered in residents of developing countries presenting with refractory chronic diarrhea, weight loss, and mesenteric lymphadenopathy.
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PMID:Immunoproliferative Small Intestinal Disease Associated with Overwhelming Polymicrobial Gastrointestinal Infection with Transformation to Diffuse Large B-cell Lymphoma. 2690 4