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Objective: Renal involvement is a common manifestation of systemic amyloidosis. Amyloid load can be predicted by histopathological grading of amyloid deposits in renal biopsy specimens. This study aimed to determine the relationship of renal amyloid deposition grade with clinical manifestations and outcomes in patients with biopsy-proven renal non-AA amyloidosis. Methods: This retrospective cohort study included 74 subjects with renal non-AA amyloidosis (52 light chain amyloidosis and 22 unclassified amyloidosis). Baseline characteristics and follow-up data were recorded. Pattern and quantity of amyloid deposition in glomeruli, interstitium, vessels, and tubulointerstitial changes were scored. Renal Amyloid Prognostic Score was obtained by addition of all scores and divided into 3 grades (Renal Amyloid Prognostic Score grades I, II, III). Results: In light chain amyloidosis group, the median follow-up was 11 (4-45) months. The baseline estimated glomerular filtration rate was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Death-censored Renal survival was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Renal Amyloid Prognostic Score grade III was a significant predictor of lower renal survival. Patient survival was not significantly different according to Renal Amyloid Prognostic Score grade. Receiving autologous stem cell transplantation treatment was associated with better patient survival. The type of amyloid could not be determined in 22 patients. In this group, baseline estimated glomerular filtration rate was significantly lower in patients with Renal Amyloid Prognostic Score grade III. Conclusions: In patients with light chain amyloidosis, baseline renal function is associated with Renal Amyloid Prognostic Score grade. Renal survival is significantly lower in patients with the highest Renal Amyloid Prognostic Score grade. However, patient survival is not significantly different according to Renal Amyloid Prognostic Score grade. [ABSTRACT FROM AUTHOR]

Objective: Systemic amyloidosis is a rare disorder, characterized by extracellular accumulation of Congo red positive fibrillar amyloid protein deposits that have an amorphous, eosinophilic appearance on hematoxylin-eosin stained preparations. The kidney is the most commonly affected organ by systemic amyloidosis. Congo red staining increases the positive birefringence of the weakly birefringent unstained amyloid. In this study, we investigated the potential diagnostic power of digitally reinforced birefringence of routine hematoxylin-eosin stained slides from renal biopsies. Material and Method: We reviewed 130 hematoxylin-eosin stained slides for polarization. Sixty-five new amyloidosis cases were diagnosed by renal biopsy. All renal biopsies were evaluated by light microscopy and immunofluorescence. Slides were reevaluated blindly using a microscope (Olympus BX51) that was attached polarization filters and connected to a digital camera (Olympus DP21, SAL). Deposits that showed green birefringence on hematoxylin-eosin preparations with digitalized microscopy were considered positive and the results were confirmed using Congo red. Results: Of the 65 Congo red confirmed amyloid positive biopsies, 61 showed green birefringence with hematoxylin-eosin. Of the 65 Congo-red confirmed amyloid negative biopsies, two were considered to be false positive. The sensitivity, specificity, and positive and negative predictive values were estimated as 94%, 97%, 97% and 94% respectively. Conclusion: We concluded that polarized hematoxylin-eosin sections can be used digitally as a fast and first step diagnostic method for renal amyloidosis. [ABSTRACT FROM AUTHOR]

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Objective: Amyloidosis represents extracellular deposition of congophilic fibrils of similar morphology but different chemical composition. The types frequently involving the kidney are AA (serum amyloid A) and AL (light chain amyloid). Hereditary amyloidoses, which can also affect the kidney, are being described with increasing frequency. Distinguishing the type of amyloid is important because of the different treatments and outcomes. Typing of amyloidosis by immunohistochemistry (IHC) is believed to be unreliable, except for AA amyloid. We reevaluated our cases in this study to draw attention to the nonAA amyloid deposits in renal biopsies with amyloidosis. Material and Method: Renal biopsies diagnosed as amyloidosis between 1997 and 2008 were stained for amyloid A with IHC. Cases with negative amyloid A staining were included in the study. Results: We found 190 cases of renal amyloidosis (198 biopsies) during the study period. Thirty cases (33 biopsies) with negative antiAA staining were studied. Among those 30 cases with nonAA amyloidosis, secondary AL amyloidosis was due to multiple myeloma in 8 cases and plasma cell dyscrasia in 2 cases. Hereditary renal amyloidosis was suspected in some cases. Conclusion: Using amyloid A IHC on a routine basis and the clinical history are essential to diagnose AA amyloidosis. The differential diagnosis of nonAA amyloidosis may be inconclusive on IHC. The diagnostic approach should be multidisciplinary and requires careful clinical and pathological evaluation. The limitation of IHC may necessitate direct typing of the amyloid protein extracted from paraffin-embedded specimens in some cases. [ABSTRACT FROM AUTHOR]