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

Renal presentation

Azotemia and proteinuria

Azotemia

Azotemia

Proteinuria

Proteinuria

 SCr (mg/dl)

2.10

1.93

1.88

1.06

1.00

 eGFR^a (ml/min/1.73 m²)

31.0

27.3

35.5

52.5

75.3

 UPCr

3.64

1.64

0.89

4.26

1.39

Hematologic Dx

MM

MM

MM

MM

MM

BM plasma cell (%)

13.2

66.5

47.3

11.4

11.0

ISS

II

n.a.

I

II

I

 Serum β2-microglobulin (μg/ml)

4.70

n.a.

3.01

4.26

2.79

 Urine β2-microglobulin (μg/ml)

5.48

n.a.

0.61

0.46

n.a.

Monoclonal Ig

κ

G-κ

λ

G-λ

A-λ

Serum κ/λ ratio^b

193.43

37.61

0.01

< 0.01

0.01

Urine κ/λ ratio^c

1245.65

284.08

0.11

< 0.01

n.a.

FS^d

Absent

Absent

Absent

Absent

Absent

 Serum uric acid (mg/dl)

5.4

7.9

8.4

3.6

8.6

 Serum phosphorus (mg/dl)

3.2

3.6

4.2

3.7

3.8

Treatment

VMP#9

VTD#5 ➔ SCT

VMP#2

VTD#3 ➔ SCT

VTD#5

Last f/u (month)

20

24

4

20

6

Hematologic outcome

PR

CR

PR

CR

PR

 SCr (mg/dl)

1.26 (WNL)

0.72 (WNL)

1.44 (improved)

0.97 (WNL)

0.87 (WNL)

 eGFR^a (ml/min/1.73 m²)

55.7 (WNL)

84.7 (WNL)

48.2 (improved)

58.0 (WNL)

88.3 (WNL)

 UPCr

0.23 (improved)

< 0.2 (WNL)

< 0.2 (WNL)

< 0.2 (WNL)

0.25 (improved)