[Non-Hodgkin lymphoma associated with a long history of protein losing enteropathy]

A 32-year-old man with an 18-year history of protein losing enteropathy (PLE) was admitted to hospital for abdominal distention. On physical examination, he had massive pleural effusion, ascites and edema of the right leg, but no superficial lymphadenopathy or organomegaly. Laboratory studies revealed mild microcytic anemia and hypoproteinemia. alpha 1-antitrypsin clearance was elevated (316 ml/day). Examination of ascites disclosed numerous lymphoblastoid cells of B cell phenotype with mu chain and lambda light chain of immunoglobulin (Ig) in the cytoplasm. Southern blot analysis showed monoclonal rearrangement of mu chain and lambda chain genes. No evidence of lymphomatous involvement of lymph nodes and non-lymphoid organs was found by CT scan, ultrasound echography and gallium scan of the chest and abdomen. Bone marrow biopsy was negative. Thus, a diagnosis of non-Hodgkin lymphoma (NHL) stage IVB limited in the pleural and peritoneal cavities was made. He was treated with the combination chemotherapy of BACOD with high dose ara-C or methotrexate followed by 4 doses of autologous LAK cell infusion resulting in no significant response. The massive pleural effusion, ascites and edema of the leg have not been improved. We consider this to be a rare case of NHL associated with PLE which is extremely resistant to chemotherapy or LAK therapy.