Clinical and Histologic Characteristics of Dysproteinemias

Clinical and Histologic Characteristics of Dysproteinemias

Clinical presentationAcute kidney injuryNephrotic syndromeNephritic syndromeNephritic syndromeNephritic syndromeNephritic or nephrotic hyperviscosity
UrinalysisBland or proteinuria (no or minimal albumin)Bland predominant albuminuriaHematuria Proteinuria castsHematuria Proteinuria castsHematuria Proteinuria castsHematuria Proteinuria casts
Serum paraprotein classIgD/IgA/IgGNoneNoneUncommonIgGIgM
Serum free light chainsκ and λλ > κ (primary)
None (secondary/hereditary)
κ > λ (LCDD)UnknownUnknownλ
Light microscopyInterstitial fibrosis, edema, inflammation, and giant cells with distal castsAll show similar features with acidophilic deposits in the mesangium, peripheral capillaries, and blood vesselsHeterogeneous most severe pattern is membranoproliferative (mesangiocapillary) with nodularityHeterogeneous mesangial to membrano-proliferative patternHeterogeneous proliferative to membrano-proliferative patternSubendothelial deposits and thrombi
Congo red stainNegativeAll positiveNegativeNegativeNegativeNegative
Immunofluorescenceκ or λ onlyλ > κκ > λPolyclonal IgG
κ and λ
Monoclonal IgGκ or IgGλ ± IgMIgM ± IgG
Complement C3NegativeAll negativeNegativePositivePositivePositive
Electron microscopyTubular crystalsAll show similar random fibrils 8–12 nmAmorphous dense depositsRandom fibrils 15–30 nmParallel microtubules 10–90 nmDense deposits ± cryoglobulin microtubules

Nephrotic syndrome = edema, hypoalbuminemia, and heavy albuminuria.Nephritic syndrome = hematuria and proteinuria, hypertension, kidney impairment.LCDD , Light chain deposition disease.


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