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Table 1 Clinical features and treatment outcomes of patients with light chain proximal tubulopathy

From: Clinicopathological characteristics of light chain proximal tubulopathy in Korean patients and the diagnostic usefulness of immunohistochemical staining for immunoglobulin light chain

 Case #1Case #2Case #3Case #4Case #5
Renal presentationAzotemia and proteinuriaAzotemiaAzotemiaProteinuriaProteinuria
Renal baseline
 SCr (mg/dl)2.101.931.881.061.00
 eGFRa (ml/min/1.73 m2)31.027.335.552.575.3
 UPCr3.641.640.894.261.39
Hematologic DxMMMMMMMMMM
BM plasma cell (%)13.266.547.311.411.0
ISSIIn.a.IIII
 Serum β2-microglobulin (μg/ml)4.70n.a.3.014.262.79
 Urine β2-microglobulin (μg/ml)5.48n.a.0.610.46n.a.
Monoclonal IgκG-κλG-λA-λ
Serum κ/λ ratiob193.4337.610.01< 0.010.01
Urine κ/λ ratioc1245.65284.080.11< 0.01n.a.
FSdAbsentAbsentAbsentAbsentAbsent
 Serum uric acid (mg/dl)5.47.98.43.68.6
 Serum phosphorus (mg/dl)3.23.64.23.73.8
TreatmentVMP#9VTD#5 ➔ SCTVMP#2VTD#3 ➔ SCTVTD#5
Last f/u (month)20244206
Hematologic outcomePRCRPRCRPR
Renal outcome
 SCr (mg/dl)1.26 (WNL)0.72 (WNL)1.44 (improved)0.97 (WNL)0.87 (WNL)
 eGFRa (ml/min/1.73 m2)55.7 (WNL)84.7 (WNL)48.2 (improved)58.0 (WNL)88.3 (WNL)
 UPCr0.23 (improved)< 0.2 (WNL)< 0.2 (WNL)< 0.2 (WNL)0.25 (improved)
  1. aBased on Modification of Diet in Renal Disease Study equation
  2. bNormal range: 0.26–1.65
  3. cNormal range: 2.04–10.37
  4. dGlycosuria, hypouricemia, hypophosphatemia, polyuria, acidosis, and hypokalemia
  5. M male, F female, SCr serum creatinine, eGFR estimated glomerular filtration rate, UPCr urine protein/creatinine ratio, Dx diagnosis, MM multiple myeloma, BM bone marrow, ISS International Staging System for Multiple Myeloma, n.a. not available, Ig immunoglobulin, FS Fanconi syndrome, VMP Velcade, Melphalan, prednisone, VTD Velcade, thalidomide, dexamethasone, SCT autologous stem-cell transplantation, f/u follow-up, PR partial remission, CR complete remission, WNL within normal limits
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