Like other forms of amyloidosis, this newly discovered type involves the improper folding of protein molecules. This improper folding leads to the harmful buildup of amyloid, which is the name for the fibrous clumps of protein molecules that then accumulate in the body. There’s some evidence that the LECT2 protein is involved with cell growth and repair, but further research is required to understand its specific function in the cell. (3) LECT2 is a unique protein in that it can form amyloid deposits even in its unmutated form, according to a 2022 review. LECT2 amyloidosis (known as ALECT2 for short) is considered a systemic form of amyloidosis, meaning it is not confined to one part of the body (thanks to the leukocyte chemotactic factor 2 circulating throughout the body). It can cause amyloid buildup in someone’s liver, spleen, colon, and adrenal glands. But in a majority of cases, ALECT2 causes the accumulation of amyloid in the kidneys (though doctors aren’t sure exactly why this happens). (2) Because another, more common form of amyloidosis — known as AL amyloidosis — can also cause kidney problems, there’s evidence that ALECT2 is sometimes misdiagnosed as the AL type. (2) This is a problem because AL amyloidosis is occasionally treated with chemotherapy — something that won’t help people with LECT2 amyloidosis, and which can be very harmful. (3) There are no set figures on the number of patients with LECT2 amyloidosis. But the disease is thought to be quite rare, accounting for approximately 3 percent of all amyloidosis cases. Men and women are affected equally. (3,4) However, it seems to be much more common among people with Hispanic ancestry. (5) Also, though doctors have diagnosed ALECT2 in patients as young as age 43, the average age of diagnosis is 66. (2) Only a very small number of people have ever been diagnosed with ALECT2 before age 50. (1) Fortunately, the prognosis for ALECT2 is better than that of AL and AA types. That said, the kidney is the most commonly involved organ in ALECT2, and when affected, ALECT2 can result in nephrotic syndrome or kidney failure if not treated. (4) RELATED: Everything You Always Wanted to Know About Cholesterol While these initial steps can help a doctor diagnose a patient with amyloidosis, more complicated testing is required to identify ALECT2. All of these tests involve closely examining the biopsied tissue to determine what type of amyloid they contain. Low-sodium diets may also be helpful because they reduce the body’s retention of water. People with the disorder are told to limit their fluid intake to about 50 ounces (1.5 liters) a day, and to spread that intake evenly throughout the day (6). In severe cases, ALECT2 patients may require dialysis (blood purification) or a kidney transplant. (2) Because ALECT2 amyloidosis is a relatively newly discovered disease, doctors are still trying to get a handle on its specific symptoms, its progression, and the best methods of treatment. (2) A lot more research is needed, but scientists and doctors are working to better understand this disease.
