Table 1.
Summary of the clinicopathological features, treatments and outcomes of the 3 patients included in this study
| Cases | Case 1 | Case 2 | Case 3 |
|---|---|---|---|
| Gender | Female | Male | Male |
| Age of onset | 48 | 55 | 65 |
| Chief Complain | edema of face and lower limbs and foamy urine for five months | edema of lower extremities for eleven months | bilateral lower limb edema for one month |
| Blood pressure | 123/78 mmHg | 169/103 mmHg | 93/56 mmHg, postural hypotension |
| Serum creatinine | 79.0 mmol/L | 215.0 mmol/L | 128.5 mmol/L |
| Serum nitrogen urea | 8.60 mmol/L | 23.00 mmol/L | 7.94 mmol/L |
| Serum albumin | 35.4 g/L | 19.8 g/L | 16.2 g/L |
| urine protein/creatinine ratio (UPCR) | 7.27 g/g Cr | 11.14 g/g Cr | 9.34 g/g Cr |
| Serum Anti-PLA2R | Positive (ELISA, Cut-off ratio ≥ 1.00) | 200.78 RU/mL (CLIA) | untested |
| Renal PLA2Ra (IHC) | untested | untested | Negative |
| SPE and sIFE | Monoclonal Immunoglobulin was not found | Monoclonal Immunoglobulin was not detected | Monoclonal Immunoglobulin was not detected |
| Serum or urine free light chain ratio (kappa/lambda) | negative | negative | Serum: 6.37 ratio (normal range 0.26–1.65) |
| Bone marrow aspiration and biopsy | Negative | a plasma cell population of 3.0%. | a clonal plasma cell population in the marrow, accounting for 3.0% of the cells |
| FCM analysis of bone marrow | did not reveal any evidence of restricted light chain expression. | no detection of monoclonal malignant plasma cells | clonal plasma cells comprised approximately 3.27% of the nuclear cells in the bone marrow |
| Method to classification diagnosis of Amloidosis | LMD/MS and WES | Immunohistochemistry | Immunofluorescence |
| Cardiac function | ultrasonic cardiogram: not notable changes, EF 64% | ultrasonic cardiogram: left ventricle hypertrophy (LVPW 12 mm), EF 64% | Cardiac MRI revealed ventricular septal thickening and patchy latent enhancement |
| Diagnosis | Nonhereditary ApoA-1-related renal Amyloidosis coexisting with PLA2R-associated MN (Stage 2) | Renal LECT2 Amyloidosis and PLA2R-associated MN (Stage 1) | Systemic AL Amyloidosis and MN (Stage 1) |
| Treatment | Prednisone and Tacrolimus |
Prednisone and cyclosporine. Cyclosporine was replaced by Rituximab because of decreased renal function |
Bortezomib and Dexamethasone Midodrine |
| Follow-up | 8 years | 3 years | sudden cardiac death happened after 4 weeks of being diagnosed |