Author/Editor		Merta, M; Ryšava, R; Špička, I; Haber, J; Honsova, E; Vernerova, Z
Title		Immunoglobulin fragment's kidney deposition in myeloma multiplex
Type		članek
Source		In: Lindič J, Kaplan-Pavlovčič S, editors. Zbornik prispevkov 1. slovenski nefrološki kongres z mednarodno udeležbo; 1996 okt 23-26; Portorož. Ljubljana: Klinični center, Nefrološka klinika,
Publication year		2000
Volume		str. 40-3
Language		eng
Abstract		Myeloma kidney often presents as classical type of kidney involvement in myeloma multiplex (MM), however renal AL - amyloidosis as well as light chain deposition disease (LCDD) represent other types of kidney lesion due to immunoglobulin fragment's kidney deposition. In AL amyloidosis fragments of the variable portion of monoclonal light chains are found as glomerular, tubular or/and vascular deposits in the kidney. AL amyloidosis develops only in a minority of cases of MM, but presents as a major therapeutical problem, since it is refractory to therapeutical efforts and in only part of multiorgan amyloidosis involvement. LCDD appears to be pathogenetically similar, except that the light chains do not form fibrils and the deposits are Congo red negative, patients typically present with the nephrotic syndrome. In order to assess occurrence of renal AL - amyloidosis, its clinical manifestations and possibilities of treatment, we analyzed data from a large group of patients with MM followed in our medical department. We investigated a group of 82 patients with monoclonal gammapathy (MG) (37 m, 45 f, mean age 63.5 +- 13.6 years). Diagnosis in majority of patients with MG was MM (59 pts, i. e. 72%), less frequent was diagnosis of Waldenstrom's macroglobulinemia, monoclonal gammapathy of uncertain significance (MGUS) and non - Hodgkin's lymphoma (23 pts, i. e. 28%). Proteinuria was detected in 66 pts. (80.5%), from this number of Bence Jones type in 54 pts. (54%) (in 32 pts kappa, in 21 pts lambda, in 1 pt. kappa + lambda). Nephrotic syndrome manifested in 5 patients (6.1%) - all patients with amyloidosis. (Abstract truncated at 2000 characters.)
Descriptors		MULTIPLE MYELOMA KIDNEY DISEASES AMYLOIDOSIS ADULT MIDDLE AGE PARAPROTEINEMIAS WALDENSTROM'S MACROGLOBULINEMIA LYMPHOMA, NON-HODGKIN'S