Other Amyloidosis
The section Other Amyloidosis is under construction. 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