A 75-year-old patient presented with fluctuating swelling around the eyes, vasculitis at the lower legs and back of the upper legs and an extreme hypogammaglobulinaemia. An extensive work-up revealed the presence of secondary immunoglobulin M cryoglobulinaemia related to a monoclonal B-cell lymphocytosis. Precipitation of proteins also resulted in a decreased C1-esterase inhibitor causing angioedema. She was treated with an elderly chronic lymphocytic leukaemia regimen consisting of obinutuzumab and chlorambucil with a subsequent clinical and haematological remission.

(BELG J HEMATOL 2019;10(2):85–8)