Association of a POEMS syndrome and light chain deposit disease: first case report

Monoclonal immunoglobulin (Ig) deposition diseases are characterized by dep osition in tissues of excessive amounts of the Ig, compromising organ funct ions. Light chain deposition disease (LCDD) and AL amyloidosis are the comm onest [Buxbaum 1992]. LCDD is usually characterized by rapidly progressive renal failure with glomerular and tubular deposits of Ig fragments mostly c omposed by kappa light chain. Monoclonal Ig production can also be observed associated with various symptoms, that, taken together, have been describe d as the Crow-Fukase syndrome or POEMS syndrome. It associates polyneuropat hy, organomegaly, endocrinopathy, monoclonal Ig, and skin changes. In POEMS syndrome, renal abnormalities are rare and are reported as a moderate rena l insufficiency with mild proteinuria or acute functional renal insufficien cy leading in some cases to end-stage renal failure [Fukatsu et al. 1991]. Although a monoclonal Ig is produced, no Ig deposit disease had been descri bed in POEMS syndrome except a case of AL amyloidosis [Toyokuni et al. 1992 ]. Here, to our knowledge, we report the first case of an LCDD associated w ith a POEMS syndrome. Although an autologous bone marrow graft was realized , the monoclonal component reappeared and was responsible for end-stage ren al disease, cachexia. and death.