Although LPL is often associated with a paraprotein, usually of the IgMtype, this is not required for the diagnosis and sometimes can be absent.(6,7) Although patients with asymptomatic disease can maintain awatchful waiting strategy, LPL with cytopenias or symptomatic disease manifested byhyperviscosity from IgM paraprotein, amyloidosis, cryoglobulinemia, central nervoussystem involvement or severe and/or advancing peripheral neuropathy should beconsidered for therapy. Here, CD40LG is linked to peripheral neuropathy.