Characteristics of the 32 study patients with Fanconi syndrome and monoclonal gammopathy
Characteristic . | Value . |
|---|---|
| Female, no. | 10 |
| Male, no. | 22 |
| Age, y, median (range) | 58 (31-81) |
| Length of follow-up, mo, mean (range) | 65 (2-238) |
| Urine protein electrophoresis, no. of patients | |
| κ light chain | 29 |
| λ light chain | 3 |
| Serum protein electrophoresis, no. of patients | |
| κ light chain | |
| IgA κ | 3 |
| IgG κ | 9 |
| IgM κ | 2 |
| Free κ | 5 |
| λ light chain | |
| IgG λ | 3 |
| Negative | 10 |
| Serum studies | |
| Hypokalemia, no. of patients (%) | 14 (44) |
| Hypophosphatemia, no. of patients (%) | 16 (50) |
| Hypouricemia, no. of patients (%) | 21 (66) |
| Initial creatinine, mg/dL, median (range) | 176.8 μM (79.56-327.08) |
| Normal 1,25-hydroxyvitamin D | 5 of 5 tested |
| Normal PTH | 10 of 12 tested |
| Elevated alkaline phosphatase | 15 of 25 tested |
| Urine studies | |
| Aminoaciduria | 30 of 30 tested |
| Glucosuria | 27 of 27 tested |
| Phosphaturia | 6 of 17 tested |
| Kidney biopsy, no. of patients | 17 |
| Cytoplasmic crystals | 8 |
| Acute tubular necrosis | 1 |
| Interstitial fibrosis | 4 |
| Nonspecific | 4 |
| Diagnosis of dysproteinemia | |
| MM | 10 |
| WM | 2 |
| SMM | 6 |
| MGUS | 14 |
| Outcome | |
| ESRD | 5 |
| Transformation from MGUS to MM | 1 |
| Death | 14 |
| Alkylator-induced AML or MDS | 4 |
Characteristic . | Value . |
|---|---|
| Female, no. | 10 |
| Male, no. | 22 |
| Age, y, median (range) | 58 (31-81) |
| Length of follow-up, mo, mean (range) | 65 (2-238) |
| Urine protein electrophoresis, no. of patients | |
| κ light chain | 29 |
| λ light chain | 3 |
| Serum protein electrophoresis, no. of patients | |
| κ light chain | |
| IgA κ | 3 |
| IgG κ | 9 |
| IgM κ | 2 |
| Free κ | 5 |
| λ light chain | |
| IgG λ | 3 |
| Negative | 10 |
| Serum studies | |
| Hypokalemia, no. of patients (%) | 14 (44) |
| Hypophosphatemia, no. of patients (%) | 16 (50) |
| Hypouricemia, no. of patients (%) | 21 (66) |
| Initial creatinine, mg/dL, median (range) | 176.8 μM (79.56-327.08) |
| Normal 1,25-hydroxyvitamin D | 5 of 5 tested |
| Normal PTH | 10 of 12 tested |
| Elevated alkaline phosphatase | 15 of 25 tested |
| Urine studies | |
| Aminoaciduria | 30 of 30 tested |
| Glucosuria | 27 of 27 tested |
| Phosphaturia | 6 of 17 tested |
| Kidney biopsy, no. of patients | 17 |
| Cytoplasmic crystals | 8 |
| Acute tubular necrosis | 1 |
| Interstitial fibrosis | 4 |
| Nonspecific | 4 |
| Diagnosis of dysproteinemia | |
| MM | 10 |
| WM | 2 |
| SMM | 6 |
| MGUS | 14 |
| Outcome | |
| ESRD | 5 |
| Transformation from MGUS to MM | 1 |
| Death | 14 |
| Alkylator-induced AML or MDS | 4 |
PTH indicates parathyroid hormone; MM, multiple myeloma; WM, Waldenström macroglobulinemia; SMM, smoldering multiple myeloma; MGUS, monoclonal gammopathy of undetermined significance; ESRD, end-stage renal disease; AML, acute myeloid leukemia; and MDS, myelodysplastic syndrome.