LIGHT-CHAIN DEPOSITION DISEASE OF THE KIDNEY - MORPHOLOGICAL ASPECTS IN 24 PATIENTS

Citation
Eh. Strom et al., LIGHT-CHAIN DEPOSITION DISEASE OF THE KIDNEY - MORPHOLOGICAL ASPECTS IN 24 PATIENTS, Virchows Archiv, 425(3), 1994, pp. 271-280
Citations number
31
Categorie Soggetti
Pathology
Journal title
ISSN journal
09456317
Volume
425
Issue
3
Year of publication
1994
Pages
271 - 280
Database
ISI
SICI code
0945-6317(1994)425:3<271:LDDOTK>2.0.ZU;2-I
Abstract
Renal biopsies and autopsy specimens of 23 patients with light chain d eposition disease (LCDD) and one with only heavy chain deposits, were studied by light (LM) and electron microscopy (EM) as well as immunohi stology (IH). Thirteen patients had multiple myeloma; 1 had lymphoma, and 1 chronic myeloid leukaemia with polycythaemia vera. In nine patie nts, no lymphoproliferative disease was identified. The LM lesions mos t suggestive of LCDD, nodular glomerulosclerosis (NS) and thickening a nd wrinkling of the tubular basement membranes (TBM), were present in only ten and 13 patients, respectively. In five of seven specimens wit hout NS or TBM thickening by LM, EM was negative, indicating a limited value of EM in confirming the diagnosis. Renal amyloidosis was not id entified, but in one patient amyloid in the heart and tongue was seen at autopsy. One patient had both granular and extensive glomerular non -amyloid fibrillary deposits. In two patients myeloma casts were ident ified. Twenty-one patients showed renal LC immune reactivity, 1 had bo th alpha heavy and lambda LC, 1 had only detectable gamma heavy chain. One biopsy was negative by IH, but had characteristic electron dense deposits. In six patients with immune reactivity to LC, no electron de nse deposits could be identified by EM. This study emphasizes the spec trum of renal changes by LM and EM in LCDD, the frequent lack of consi stency between deposits detected by IH and EM and the difficulty in co ming to a definite diagnosis without LM, EM and IH. The results of thi s study and examination of the literature indicates that extensive mor phological changes are more often present in kappa than in lambda LCDD .