RENAL-DISEASE AND PATIENT SURVIVAL IN LIGHT-CHAIN DEPOSITION DISEASE

We evaluated retrospectively the presenting clinical features, respons e to treatment and clinical course of 19 patients with LCDD, 11 of who m had multiple myeloma. At presentation, renal insufficiency was prese nt in 18 patients and proteinuria in 16. Renal biopsy revealed typical LCDD in 16 patients, while in the remaining three LCDD was associated with other abnormal tissue deposits. Extrarenal signs were observed i n 12 patients (63%), with the liver, heart and peripheral nerves being the most frequently involved organs. After diagnosis, 18 patients und erwent therapy: 2 received steroids alone and 16 were treated with ste roids and cytotoxic drugs; 7 patients also underwent plasma exchange. At the end of the first month of treatment renal function improved in 5 patients, worsened in 5 and remained unchanged in 8. All but 3 of th e patients continued treatment beyond the first month: 7 patients deve loped end-stage renal disease, 5 an improvement and 4 a worsening in r enal function. No effect on proteinuria was observed. Extrarenal sympt oms developed in 4 previously unaffected patients and in 3 others they extended to more organs. Sixteen patients died: 12 during the first y ear of the follow-up, and 4 at 21st, 34th, 37th and 82nd month of obse rvation. Five patients died from neoplastic cachexia, 4 from hypokinet ic cardiopathy, 3 from hemorrhagic complications, 2 from pneumonia and one from unknown cause. Mean patient survival after presentation was 18.1 +/- 20.7 months. In our patients clinical outcome was generally v ery poor, and it was probably influenced by the high prevalence of mul tiple myeloma, the multiorgan involvement of extrarenal disease and th e presence of different types of deposits in some patients. In conclus ion, LCDD in patients with renal disease is very serious, extrarenal i nvolvement is frequent and therapy has beneficial effects only in a mi nority of patients.