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Table 2 Organ tropism of AL amyloidosis related to IgL germline genes

From: Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications

Light chain germline gene

Prevalence in AL (%) [28, 87, 88]

Organ tropism

Other associated features

κ light chain

~ 20

↑ Hepatic involvement [28, 79, 87, 90]

Better survival outcome after ASCT [90]

  

↑ Soft tissue involvement [85]

 
  

↑ Bone involvement [85]

 

IGKV1-05

2

May ↓ renal involvement [87]

Inferior overall survival [87]

  

May ↑ advanced cardiac disease [87]

 

IGKV1-33

5

↑ Hepatic involvement [87]

High circulating dFLC [87]

  

May ↓ peripheral nerve involvement [87]

 

IGKV3-20

2

Localized AL amyloidosis [87]

–

IGKV4-01

5

↑ Gastrointestinal involvement [88]

 
  

↑ Soft tissue involvement [88]

 

λ light chain

~ 80

  

IGLV1-44

6

↑ Cardiac involvement [81, 82, 86, 87]

↓ Rate of trisomies [87]

  

May ↑ multisystem involvement [81, 82]

 
  

↓ Hepatic involvement [88]

 

IGLV2-08

3

↑ Lung involvement [88]

Associated with IgM light chain amyloidosis [88]

IGLV2-14

9

↑ Gastrointestinal localized AL [87]

↑ Intact immunoglobulin [87]

  

May ↑ cardiac involvement [82]

↓ Circulating dFLC [87, 88]

  

May ↑ peripheral nerve involvement [87], but another study did not support this finding [88]

Associated with IgM light chain amyloidosis [88]

  

May ↑ multisystem involvement [82]

 

IGLV3-01

9

May ↑ cardiac involvement [82]

–

  

May ↑ multisystem involvement [82, 84]

 
  

May ↓ advaced cardiac disease [86, 87]

 
  

May ↓ renal involvement [87]

 

IGLV3-19

2

May ↑ cardiac involvement [87]

 

IGLV3-21

5

↓ Renal involvement [87]

↑ Rate of monosomy 13q [87]

IGLV6-57

13

↑ Renal involvement [81, 82, 84, 87, 88]

↑ Rate of t(11;14) [87]

  

May ↓ cardiac involvement [14, 86]

↓ Rate of trisomies [87]

   

Better survival outcome after ASCT [81]

  1. The contents highlighted in bold indicate that they are supported by 2 or more evidences with statistical significance