Other2019-11-15T14:08:44+00:00

Other Amyloidosis

The section Other Amyloidosis is under construction and will be completed by the end of the year. A summary is presented below.

Introduction

Other amyloidosis that are of importance are:

Lect 2 Amyloidosis (ALect)

  • LECT 2 is protein first described in 1996
    • It is a chemotactic factor for neutrophils
  • ALECT 2 was first described 2014
    • It was detected with the advent of mass spectrometry amyloid typing
    • It causes kidney and liver amyloidosis
  • Almost 100% cases have renal disease
    • Usually presenting with subnephrotic proteinuria
    • And associated with a mean drop in eGFR of 0.5 ml/min/1.73 m2 per month
  • In the US it is the second to third most common form of renal amyloidosis in the US and is associated with Hispanic Heritage
  • Currently, there are five identified cases in Australia

Beta2-Microglubulin amyloidosis

  • Beta2 -microglobulin is the light-chain component of the major histocompatibility complex (MHC)
    • this form of amyloidosis is usually associated with dialysis
  • Beta2 -microglobulin is normally catabolized in the kidney after it is displaced from the MHC in the proximal tubules
    • in patients with decreased renal clearance the serum level of the beta2 -microglobulin can be much more concentrated
    • This can lead to beta2 -microglobulin amyloidosis mainly affecting the joints causing osteoarticular destruction but also muscle, tendon, ligament and sometimes gastrointestinal tract deposition
  • The prevalence has decreased with the use of high-flux biocompatible membranes, which provide better clearance of beta2 -microglobulin