Amyloid cast nephropathy: A rare presentation of multiple myeloma–associated light chain amyloidosis

Introduction: Monoclonal immunoglobulins can cause a variety of histologic patterns of kidney injury, depending on the physicochemical properties. Multiple myeloma manifests more often as light-chain nephropathy. On the other hand, light-chain amyloidosis leads to glomerular and vascular amyloid deposits, but a less common presentation with amyloid casts has also been described. Rarely, more than one histologic pattern can be present in the same patient. Case report: We report a case of a 73-year-old man, diagnosed with multiple myeloma that 8 months after achieving partial response to chemotherapy develops acute kidney injury and nephrotic syndrome. Kidney biopsy revealed features of light-chain nephropathy, amyloid cast nephropathy, and glomerular and vascular amyloid deposits. Immunofluorescence was positive for IgA (++) and lambda chains (+++) and negative for kappa chains. After the diagnosis of multiple myeloma-associated light-chain amyloidosis, chemotherapy was initiated; unfortunately, the patient died 1 month after the diagnosis. Conclusion: Amyloid casts, isolated or accompanied by other renal or extra-renal amyloid deposits, are another form of tubular toxicity caused by dysproteinemias and should be systematically screened in kidney biopsies.

Nomogram Model for Dynamic and Individual Prediction of Cardiac Response and Survival for Light Chain Amyloidosis in 737 Patients With Cardiac Involvement

ObjectiveLight chain amyloidosis (AL) with cardiac involvement is associated with poor prognosis. The existing prognostic assessment system does not consider treatment-related factors, and there is currently no effective system for predicting the response. The purpose of this study was to build an individualized, dynamic assessment model for cardiac response and overall survival (OS) for AL patients with cardiac involvement.MethodsThe records of 737 AL patients with cardiac involvement were collected through cooperation with 18 hospitals in the Chinese Registration Network for Light-chain Amyloidosis (CRENLA). We used univariate and multivariate analyses to evaluate the prognostic factors for OS and cardiac response. Then, two nomogram models were developed to predict OS and cardiac response in AL patients with cardiac involvement.ResultsA nomogram including four independent factors from the multivariate Cox proportional hazards analysis—Mayo staging, courses of treatment, hematologic response, and cardiac response—was constructed to calculate the possibility of achieving survival by adding all the points associated with four variables. The higher the score, the more likely death would occur. The other nomogram model included the courses of treatment, hematological response, and different treatment regimens, and was correlated with cardiac response. The higher the score, the more likely a cardiac response would occur.ConclusionIn conclusion, based on the large Chinese cohort of patients with AL and cardiac involvement, we identified nomogram models to predict cardiac response and OS. These models are more individualized and dynamic, and therefore, they have important clinical application value.

Electrocardiogram Characteristics and Prognostic Value in Light-Chain Amyloidosis: A Comparison With Cardiac Magnetic Resonance Imaging

Background: An electrocardiogram (ECG) is a simple and cheap non-invasive tool that shows various abnormalities and has prognostic value for patients with light-chain amyloidosis (AL). The present study aimed to explore the association between ECG characteristics and cardiac magnetic resonance (CMR)-detected amyloid burden and to investigate the prognostic value of ECG in AL amyloidosis.Methods: We prospectively enrolled 99 patients with AL amyloidosis (56 male patients; median age, 58 y). Detailed clinical information, 12-lead ECG, and CMR data were collected. All patients were followed up longitudinally, and the endpoint was all-cause mortality. ECG characteristics were analyzed and correlated with the degree of late gadolinium enhancement (LGE) and extracellular volume (ECV) by T1 mapping on CMR. The prognostic value of ECG characteristics was analyzed using Kaplan–Meier survival analysis and multivariate Cox regression.Results: During a median follow-up period of 33 months, 69 of the 99 patients died. Fragmented Q wave-R wave-S wave (QRS), pathological Q waves, the Sokolow index, QRS duration, and voltages were significantly associated with the extent of LGE, native T1, and ECV by CMR (p < 0.05). Fragmented QRS and Sokolow index showed independent prognostic value in AL amyloidosis (p = 0.001; p = 0.026, respectively). Fragmented QRS remained independent after adjusting for clinical values (hazard ratio: 2.034; 95% confidence interval: 1.148–3.603; p = 0.015). However, no ECG characteristics were independent predictors for prognosis in AL amyloidosis when LGE and ECV were included in the multivariate analysis.Conclusion: ECG abnormalities showed significant association with CMR indicators of amyloid burden. Fragmented QRS has an independent prognostic value in AL amyloidosis and could be used as an alternative marker when CMR is not available.

110 Sex-related differences in clinical presentation and outcome of patients with cardiac transthyretin amyloidosis and light chain amyloidosis

Aims We aimed to ascertain whether sex-related differences are relevant to the pathophysiology, presentation, and outcomes in different types of cardiac amyloidosis—a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Methods and results Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Wt-ATTR women, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men. Otherwise, bio-humoural parameters, NYHA class, and ECG characteristics were similar. No differences in outcome were observed with regard to sex. Conclusions In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, outcome was not affected. Nevertheless, women with wtATTR showed a worse profile at diagnosis and evidence suggesting a later recognition of disease compared to men, highlighting the need for a higher index of suspicion in female patients.