Retrospective clinical and morphological analysis of patients with AL amyloidosis (the 2008 to 2015 nephrobiopsies)

Arkh Patol. 2020;82(6):29-35. doi: 10.17116/patol20208206129.


OBJECTIVE: To study correlations between the clinical signs of dysfunction and pathological structural changes in the renal parenchyma in a group of Russian patients with AL amyloidosis in 2008-2015.

SUBJECTS AND METHODS: A total group (At) including Group 1 with AL (kappa + lambda light chains) (n=46) was divided into subgroups: 2λ) 40 patients with AL-lambda (AL-λ); 3κ) 6 patients with AL-kappa (AL-κ). All the patients underwent standard laboratory and instrumental studies: determinations of the peak systolic and diastolic blood pressures (SBP and DBP, respectively, mm Hg), glomerular filtration rate (GFR) (ml/min/1.73 m2) by the EPI equation, daily protein loss (g/day). Polyclonal antibodies against kappa and lambda light chains, AA component, and transthyretin (DAKO, Denmark) were used as immunomorphological markers. Light optical structural changes were semiquantitatively assessed, by ranking the following analyzed sign: interstitial focal sclerosis (FS), tubular atrophy (TA), interstitial inflammatory infiltration (II) semi-quantitatively (0 – no; 1 – < 25%; 2 – <50%, 3 – >50% of the volume of a histological compartment). Glomerulosclerosis (GS) was defined as the percentage of sclerotic glomeruli. The extent of amyloid depositions in the renal parenchyma structures was estimated according to the procedure proposed by Ying Yao et al., 2013.

RESULTS: The AL group showed a female preponderance (65.21%). The patients’ mean age was 62±11 years. There were no significant differences in daily proteinuria and the levels of serum creatinine, GFR, SBP, and DBP between the groups. The predominant clinical manifestation in the patients was nephrotic syndrome. A comparative analysis of the pathomorphological criteria for the spread of amyloid masses and the markers of fibroplastic processes revealed no statistically significant differences in the studied groups. Correlation analysis of the spread of AL deposits in the renal parenchyma in the patients of Group 1 and Subgroup 2λ, as well as laboratory data showed that there were significant (p<0.05) correlations with GFR, serum creatinine, unlike in Subgroup 3κ. At the same time, the analysis demonstrated that daily proteinuria had a significant positive correlation with VA, IA, GA, and TA values in Subgroup 3κ, unlike in Group 1 and Subgroup 2λ. Positive correlations were found between glomerulosclerosis and VA in Subgroup 2 λ and IA in Group 1. Sclerotic (FS and TA) changes in the tubular interstitium (TIN) were significantly positively correlated with all the indicators of AL (GA, VA, IA, TA) in the examinees in Group 1 and Subgroup 2λ, but not in Subgroup 3κ. Inflammatory TIN infiltration showed statistically significant (p<0.05) positive correlations with IA and VA in Group 1 and Subgroup 2λ and their absence in subgroup 3κ.

CONCLUSION: : The retrospective analysis of nephrobiopsy specimens from of patients with AL amyloidosis revealed that kidney damage was mainly associated with the development of λ-associated AL amyloidosis. The clinical and laboratory parameters were correlated with the pathomorphological criteria for loading the renal parenchyma with amyloid masses. The findings suggest that there are clinical and morphological features of the subclasses of AL amyloidosis, which may be of value for predicting the course and progression of the disease.

PMID:33274623 | DOI:10.17116/patol20208206129