Immunoproliferative Small Intestinal Disease

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75 relevant articles (10 outcomes, 8 trials/studies) found for this Disease

Description: A condition that is caused by HYPERPLASIA of LYMPHOCYTES in the small intestine (INTESTINE, SMALL) and the mesenteric LYMPH NODES. These lymphocytes produce an anomalous alpha heavy chain protein. Generally, these IPSID patients have either concurrent LYMPHOMA or develop lymphoma within a few years. The disease was first described in the Mediterranean region and is characterized by malabsorption; WEIGHT LOSS; DIARRHEA; and STEATORRHEA.

Also Known As:

Disease, alpha-Chain; Diseases, alpha-Chain; Mediterranean Lymphoma; alpha Chain Disease; alpha-Chain Diseases; Heavy Chain Disease, IgA Type; IPSID; Lymphoma, Mediterranean; alpha-Chain Disease

Relationship Network

Disease Context: Research Results

Neoplasms: 415397

Neoplasms by Histologic Type: 1

Lymphoma: 23766

Immunoproliferative Small Intestinal Disease: 75

Digestive System Diseases: 31

Gastrointestinal Diseases: 1230

Gastrointestinal Neoplasms: 1591

Intestinal Neoplasms: 228

Immunoproliferative Small Intestinal Disease: 75

Intestinal Diseases: 601

Intestinal Neoplasms: 228

Immunoproliferative Small Intestinal Disease: 75

Digestive System Neoplasms: 54

Gastrointestinal Neoplasms: 1591

Intestinal Neoplasms: 228

Immunoproliferative Small Intestinal Disease: 75

Hemic and Lymphatic Diseases

Lymphatic Diseases: 1961

Lymphoproliferative Disorders: 2359

Heavy Chain Disease: 130

Immunoproliferative Small Intestinal Disease: 75

Hematologic Diseases: 1480

Blood Protein Disorders: 13

Paraproteinemias: 837

Heavy Chain Disease: 130

Immunoproliferative Small Intestinal Disease: 75

Immune System Diseases: 1319

Immunoproliferative Disorders: 26

Lymphoproliferative Disorders: 2359

Immunoproliferative Small Intestinal Disease: 75

Paraproteinemias: 837

Heavy Chain Disease: 130

Immunoproliferative Small Intestinal Disease: 75

Related Diseases

1.	Lymphoma (Lymphomas)
2.	Mumps
3.	Non-Hodgkin Lymphoma (Lymphosarcoma)
4.	Immunoproliferative Small Intestinal Disease
5.	Heavy Chain Disease

Experts

1.	Sinha, Saroj-Kant: 1 article (06/2006)
2.	Singh, Kartar: 1 article (06/2006)
3.	Kumari, Neeraj: 1 article (06/2006)
4.	Vaiphei, Kim: 1 article (06/2006)
5.	Nagi, Birinder: 1 article (06/2006)
6.	Dutta, Usha: 1 article (06/2006)
7.	Joshi, Kusum: 1 article (06/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Immunoproliferative Small Intestinal Disease:

1.	Anti-Bacterial Agents (Antibiotics)IBA 03/15/2005 - "Early-stage IPSID responds to antibiotics (30%-70% complete remission). " 01/01/1978 - "[Complete clinical, histological and immunological remission in a patient with alpha-chain disease treated by oral antibiotics (author's transl)]" 04/03/1976 - "Letter: Alpha-chain disease cured with antibiotics." 10/25/1975 - "Letter: Alpha-chain disease cured with antibiotics." 01/15/2004 - "RESULTS: Analysis of frozen intestinal tissue obtained from an index patient with immunoproliferative small intestinal disease who had a dramatic response to antibiotics revealed the presence of Campylobacter jejuni. " Order ALL the reference details at left...
2.	Tetracycline (Achromycin)FDA LinkGeneric 01/01/2001 - "This raises doubt on efficacy of tetracycline alone in treatment of IPSID. " 07/01/1998 - "Immunoproliferative small intestinal disease in a 16-year-old boy presenting as severe malabsorption with excellent response to tetracycline treatment." 03/01/1995 - "Combination chemotherapy with tetracycline and aggressive supportive care for immunoproliferative small-intestinal disease lymphoma." 05/01/1989 - "Tetracycline was necessary for the treatment of IPSID-associated diarrhea and malabsorption in spite of cytotoxic chemotherapy." 07/01/1997 - "CONCLUSIONS: The COPP regimen, with its acceptable toxicity, appears to be a good alternative as a first-line treatment for patients with Stage B or C IPSID with low grade lymphoma whereas tetracycline appears to be the initial treatment of choice for patients with Stage A disease." Order ALL the reference details at left...
3.	AntigensIBA 06/01/1985 - "These results support the suggestion that Mediterranean lymphoma is a tumour of follicle centre cells which undergoes plasma cell differentiation as a result of exposure to lumenal antigen." 03/01/1979 - "The high rate of association of both AW19 and B12 antigens in patients as compared with controls suggests a possible haplotype (Aw19, B12) association with IPSID." 01/01/1995 - "Flow cytometric analysis of DNA content, S phase fraction, and quantitative analysis of the proliferation-associated nuclear antigen, P105, were performed in 23 patients with IPSID to determine if they could be used as prognostic indicators in this disease. " 01/01/1995 - "Flow cytometric measurement of proliferation-associated nuclear antigen P105 and DNA content in immuno-proliferative small intestinal disease (IPSID)." 09/30/1978 - "Patients with Mediterranean lymphoma (M.L.) had a significant reduction in humoral immunity (IgG and IgM) as well as impaired cellular immunity (50% were anergic to three antigens--P.P.D., mumps, and dinitrochlorobenzene). " Order ALL the reference details at left...
4.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 07/01/1982 - "The alpha-chain disease protein was further purified and classified as subclass 1. The patient had a good clinical response to cyclophosphamide and prednisone, but still has intraabdominal lymphoma with gastric involvement, and his serum alpha-chain protein persists. " 09/01/2006 - "Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured." 07/01/1994 - "Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). " Order ALL the reference details at left...
5.	Vincristine (Oncovin)FDA LinkGeneric 09/01/2006 - "Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured." 07/01/1994 - "Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). " Order ALL the reference details at left...
6.	Prednisolone (Predate)FDA LinkGeneric 09/01/2006 - "Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured." 07/01/1994 - "Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). " Order ALL the reference details at left...
7.	Immunoglobulin G (IgG)IBA 01/01/1980 - "In ulcerative colitis and Crohn's disease, the IgG-cell response is very striking, In alpha-chain disease, the lamina propria is infiltrated with IgA cells that lack light-chain-producing capacity but show some ability to synthesize J chains; dimers of alpha chain are hence transported to the intestinal lumen but cannot function as antibodies. " 09/30/1978 - "Patients with Mediterranean lymphoma (M.L.) had a significant reduction in humoral immunity (IgG and IgM) as well as impaired cellular immunity (50% were anergic to three antigens--P.P.D., mumps, and dinitrochlorobenzene). " Order ALL the reference details at left...
8.	Sulfasalazine (Azulfidine)FDA LinkGeneric 12/01/1980 - "The patient was treated with sulfasalazine and melphalan, but it is not clear that the clinical remission and the subsequent disappearance of the alpha-chain disease protein should be ascribed to this treatment. " Order ALL the reference details at left...
9.	Melphalan (Alkeran)FDA Link 12/01/1980 - "The patient was treated with sulfasalazine and melphalan, but it is not clear that the clinical remission and the subsequent disappearance of the alpha-chain disease protein should be ascribed to this treatment. " Order ALL the reference details at left...
10.	Mebendazole (Vermin)FDA Link 03/01/1999 - "Reported here is a 20-year old man with IPSID lymphoma who responded to anti-malignant chemotherapy initially, but later deteriorated due to Strongyloides stercoralis infestation, which was treated successfully with mebendazole. " Order ALL the reference details at left...

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2008 - "He was diagnosed with relapsed IPSID and salvage chemotherapy was started. " 07/01/1994 - "CONCLUSION: Intensive chemotherapy produces long-term disease-free survival in locally advanced non-IPSID PSIL." 07/01/1994 - "The overall 5-year survival and disease-free survival rates for non-IPSID patients who were treated with chemotherapy were 59% and 54%, respectively. " 03/01/1999 - "Reported here is a 20-year old man with IPSID lymphoma who responded to anti-malignant chemotherapy initially, but later deteriorated due to Strongyloides stercoralis infestation, which was treated successfully with mebendazole. " 03/01/1996 - "Accordingly, although traditional antineoplastic treatments, such as surgery and radiation, are still important for the treatment of primary GI lymphomas, antibiotics may be the first line of therapy for low-grade gastric MALToma, and they are often used alone or in combination with chemotherapy for IPSID. " Order ALL the reference details at left...
2.	Laparotomy 04/15/1988 - "The results of initial exploratory laparotomy performed in 38 cases of IPSID (17 ACD and 21 NS-IPSID) are described here, and show notable similarities between the two groups: the extensive pattern of the abnormal cell infiltrate along the major part of the small intestine; frequent and extensive involvement of the mesenteric lymph nodes; the existence of several degrees of severity in small intestinal mesenteric lymph nodes; and other intraabdominal organ involvement. " 04/15/1988 - "Results of laparotomy in immunoproliferative small intestinal disease." 03/01/1989 - "Biopsy specimens taken during exploratory laparotomy provided the material for a pathological study of 23 cases of nonsecreting immunoproliferative small intestinal disease (Mediterranean lymphomas without alpha-chain disease). " 11/01/1987 - "No patient with immunoproliferative small intestinal disease developed immunologically demonstrated alpha-chain disease or frank lymphoma, when this was not found initially at explorative laparotomy." Order ALL the reference details at left...
3.	Combination Drug Therapy (Combination Chemotherapy) 03/01/1995 - "Combination chemotherapy with tetracycline and aggressive supportive care for immunoproliferative small-intestinal disease lymphoma." 07/01/1994 - "Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). " Order ALL the reference details at left...
4.	Transplants (Transplant) 07/01/1994 - "Thirty-nine patients had non-IPSID, including one with postrenal transplant lymphoma. " Order ALL the reference details at left...
5.	Pancreaticoduodenectomy 01/01/2007 - "We herein report a rare case of a massive upper gastrointestinal (GI) bleeding, caused by high-grade diffuse B-cell lymphoma of the duodenum, secondary to immunoproliferative small intestinal disease (IPSID) and treated with an emergency partial pancreatoduodenectomy. " Order ALL the reference details at left...

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