title: [Intestinal non-Hodgkin lymphoma: "immunoproliferative small intestinal disease"].
abstract: Immunoproliferative small intestinal disease (IPSID), also known as alpha chain disease, is a rare disease. In the recent WHO classification of hematopoietic and lymphoid tissue, IPSID is considered as a variant of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma. Campylobacter jejuni is a specific pathogen, found to be related to IPSID. Diagnosis is based on histology and immunochemistry (± fluorescent in situ hybridization), with presence of many variable levels of abnormal immunoglobulin in the serum, identified to be truncated alpha-heavy chains. Early-stage disease is treated by antibiotics (tetracyclines). Chemotherapy is recommended up front for patients with advanced disease at presentation or refractory to antibiotics. The chemotherapy schedule used is the CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen.
authors: Institut national d'oncologie, service d'oncologie médicale, Rabat 10100, Maroc. mrabti_h@yahoo.fr
authors: Hind
authors: H
authors: Mrabti
authors: Ghizlane
authors: G
authors: Raiss
authors: Soundouss
authors: S
authors: Raissouni
authors: El Mehdi
authors: el M
authors: Tazi
authors: Hanane
authors: H
authors: Inghaouen
authors: Ibrahim
authors: I
authors: El Ghissassi
authors: Hassan
authors: H
authors: Errihani
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D002169:Campylobacter Infections / Q000188:drug therapy / Q000473:pathology; D016123:Campylobacter jejuni; D003937:Diagnosis, Differential; D006801:Humans; D007161:Immunoproliferative Small Intestinal Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D007413:Intestinal Mucosa / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D009367:Neoplasm Staging; D011379:Prognosis; D000069283:Rituximab; D013754:Tetracyclines / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D013754:Tetracyclines; D000069283:Rituximab