Immunoproliferative Small Intestine Disease (IPSID): A Case Report

Immunoproliferative Small Intestinal Disease (IPSID) is the syndrome associated with Mediterranean lymphoma (a rare form of non-Hodgkin’s lymphoma). Many of the patients diagnosed with secretory IPSID have variable level of abnormal immunoglobulins in serum or other bodily fluids, identified as truncated alpha heavy chain globulins. Most cases are characterized by a loss of ability to synthesize light chains. As such, IPSID has been classified as a heavy chain disorder B-cell lymphoma. We present here the case of a 12-year-old boy admitted in our department for edema, abdominal pain and Failure to Thrive (FTT), in whom we suspected the diagnosis of IPSID.