Lymphomes malins non hodgkiniens de l’intestin grêle de type « immunoproliferative small intestinal disease »

2011 ◽  
Vol 40 (11) ◽  
pp. 995-1000 ◽  
Author(s):  
Hind Mrabti ◽  
Ghizlane Raiss ◽  
Soundouss Raissouni ◽  
El Mehdi Tazi ◽  
Hanane Inghaouen ◽  
...  
Keyword(s):  
Intestinal Disease ◽  
Immunoproliferative Small Intestinal Disease ◽  
Small Intestinal ◽  
Small Intestinal Disease

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Blood ◽  
2005 ◽  
Vol 105 (6) ◽  
pp. 2274-2280 ◽  
Author(s):  
Tahseen Al-Saleem ◽  
Hamid Al-Mondhiry
Keyword(s):  
B Cell ◽  
Plasma Cell ◽  
Heavy Chain ◽  
Early Stage ◽  
B Cell Lymphoma ◽  
Intestinal Disease ◽  
Molecular Features ◽  
Immunoproliferative Small Intestinal Disease ◽  
Small Intestinal ◽  
Small Intestinal Disease

AbstractImmunoproliferative 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 α heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the variable heavy chain (VH) and the constant heavy chain 1 (CH1) sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of CH1 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.

scholarly journals HLA AW19, B12 in immunoproliferative small intestinal disease.

Gut ◽  
1979 ◽  
Vol 20 (3) ◽  
pp. 226-228 ◽  
Author(s):  
B Nikbin ◽  
M Banisadre ◽  
F Ala ◽  
A Mojtabai
Keyword(s):  
Intestinal Disease ◽  
Immunoproliferative Small Intestinal Disease ◽  
Small Intestinal ◽  
Small Intestinal Disease

Primary small-intestinal lymphomas in Taiwan: immunoproliferative small-intestinal disease and nonimmunoproliferative small-intestinal disease.

1994 ◽  
Vol 12 (7) ◽  
pp. 1375-1382 ◽  
Author(s):  
L Y Shih ◽  
S J Liaw ◽  
P Dunn ◽  
T T Kuo
Keyword(s):  
Combination Chemotherapy ◽  
Disease Free Survival ◽  
Large Cell ◽  
Intestinal Disease ◽  
Primary Tumor Site ◽  
Free Survival ◽  
Immunoproliferative Small Intestinal Disease ◽  
Small Intestinal ◽  
Disease Free ◽  
Small Intestinal Disease

PURPOSE The clinicopathologic findings in 45 adult Chinese patients with primary small-intestinal lymphoma (PSIL) are described and compared with those in Western countries and in underdeveloped nations. The efficacy of combination chemotherapy is also assessed. PATIENTS AND METHOD Six patients had immunoproliferative small-intestinal disease (IPSID) indicated by the presence of alpha-heavy chain protein (alpha-CP) in body fluids or tumor tissues. Thirty-nine patients had non-IPSID, including one with postrenal transplant lymphoma. Thirty-three non-IPSID patients received a minimum of four cycles of combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). RESULTS All IPSID patients presented with the clinical and laboratory features of severe intestinal malabsorption, and all had diffuse lymphoplasmacytic infiltration in the mucosa of the small bowel. Lymphomas were localized mainly in the jejunum and mesenteric nodes. The histologic subtypes were diffuse large cell in two, immunoblastic in three, and diffuse mixed in one. All patients responded poorly to chemotherapy, with a median survival duration of 10.5 months. The common presenting symptoms of the 39 non-IPSID patients included abdominal pain (90%), weight loss (31%), abdominal mass (26%), obstruction (26%), and perforation (23%). Diffuse large-cell and immunoblastic lymphomas constituted 82% of cases. Four patients had stage IE, 19 stage II 1E, and 16 stage 112E disease according to the Musshoff's criteria; 22 had bulky tumors and 19 had multiple tumors. The tumors were completely resected in 14 patients. Of 33 patients treated with combination chemotherapy, 73% achieved a complete remission. With a median follow-up duration of 90 months, there have been four relapses, with only one at the primary tumor site. The overall 5-year survival and disease-free survival rates for non-IPSID patients who were treated with chemotherapy were 59% and 54%, respectively. CONCLUSION Intensive chemotherapy produces long-term disease-free survival in locally advanced non-IPSID PSIL.

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