Classically Light chain deposition disease(LCDD) presents
with proteinuria ( almost in the nephrotic range). A recent article in NDT looks at a small cohort
of patients that presented with LCDD with <0.5gm of proteinuria. They present 14 cases, average proteinuria
was 0.3g/day. Most had CKD at baseline.
IgG kappa was the most common light chain followed by IgM kappa. Only 3 patients had diagnostic myeloma, 2 had
WM. Interestingly
5 had MGUS and 4 had smoldering myeloma.
Serum creatinine decreased in most cases after chemotherapy was
introduced. Proteinuria never became nephrotic range. This is thus far the largest series of such
LCDD without proteinuria. Early identification of this atypical variant of LCDD
is important. It seems also that it’s a smoldering
form just like the hematologic counterpart and most of these patients had
either MGUS or SM and not full blown MM.
What did the pathology show?- diffuse tubular basement membrane thickening;
some with duplication. Rare casts were
noted. Nodular sclerosis of GN lesions
only in 2 cases and others did have some mesangial hypertrophy and ischemic lesions. No amyloidosis was noted in any cases.
Classically thought to be a proteinuric variant of myeloma, LCDD can also present as a non proteinuric disease mainly involving tubules.
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