Waldenström's macroglobulinaemia secreting a paraprotein with lupus anticoagulant activity: Possible association with gastrointestinal tract disease and malabsorption

Abstract

A 51 year old man with Waldenström's macroglobulinaemia presented with a malabsorptive syndrome related to extensive small bowel lymphangiectasia caused by mimunoglobulin accumulation. The patient's plasma had strong lupus anticoagulant activity and the IgM k paraprotein displayed specificity for the negatively charged phospholipids phosphatidyl serine and phosphatidyl inisitol, as well as the neutral phosphatidic acid. Despite treatment for the macroglobulinaemia the patient died and at necropsy was found to have myocardial ischaemia and segmental infarcts in the spleen and kidney. The coexistence of these relatively rare findings suggests a possible association between Waldenström's macroglobulinaemia with gas-trointestinal manifestations and para-protein specificity for phospholipid.