Short notice and a short but important article describing a series of patients with a relatively recently recognized subtype of amyloidosis: ALect2.
Here is a link to the full text of the article:
http://www.bloodjournal.org/content/bloodjournal/123/10/1479.full.pdf
For anyone unable to access the article via that link, email me at zonderj@gmail.com and I can send you the pdf.
The article we are reviewing this week discusses a series of 130 patients with hepatic #amyloidosis analyzed with tandem mass spectrometry at the Mayo Clinic.
Key Points:
- The most common form of hepatic amyloidosis was AL (light chain) subtype. Alect2 also commonly affects the kidneys.
- Leukocyte cell-derived chemotaxin 2 associated amyloidosis (ALect2) was next most common (25% of analyzed cases).
- ALect2 was originally observed predominantly in people of hispanic (specifically Mexican) heritage, but has since been recognized in Egyptians and First Nation People. There is also a published case report of a Punjabi patient from India with Alect2.
- No definite amyloid-associated LECT2 gene mutation has been identified, but 10 of 10 sequenced patients in another report were homozygous for the G nucleotide in a non-synonymous SNP amongst people of Mexican heritage.
We'll talk about Alect2 and other forms of hepatic and renal amyloidosis this Wednesday - join us!
