NATURAL-KILLER-CELL LYMPHOPROLIFERATIVE DISEASE-ASSOCIATED WITH NEUROPATHY

Large granular lymphoproliferative disease (LGLPD) is a heterogeneous disorder, which can be divided into two major types based on expressio n of the T-cell antigen receptor. The majority of cases represent prol iferations of cytotoxic T cells (CD3+, CD8+) with a monoclonal rearran gement of the T cell antigen receptor, but more rarely, proliferations of true natural killer cells (CD3-, CD2+, CD16+, or CD56+) are found. (1) The clinical course of LGLPD is usually indolent and is most commo nly associated with cytopenia, recurrent infection, and rheumatoid art hritis.(1) We now describe 2 patients with chronic natural killer (NK) cell LGLPD who initially presented with a sensory-motor neuropathy an d peripheral nerve lymphoid infiltration. Symptomatic improvement has occurred in these 2 patients after lymphocytotoxic therapy, with a con current decline in circulating natural killer cells.