[Intestinal non-Hodgkin lymphoma: "immunoproliferative small intestinal disease"]. / Lymphomes malins non hodgkiniens de l'intestin grêle de type "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.