Leukocyte Chemotactic Factor 2 (LECT2) amyloidosis, or ALECT2, is a rare form of renal amyloidosis caused by the buildup of abnormally folded LECT2 proteins in the kidney. Over time this buildup can reduce kidney function and eventually may result in kidney failure.
ALECT2 history, risk factors, and prevalence
ALECT2 was only recently discovered with the first case being diagnosed in 2008. This does not mean that people didn’t get ALECT2 before 2008, only that it was not recognized by doctors before that time.
Although ALECT2 is rare in the general population, it appears to be more common in people of certain ethnicities, including:
- Hispanic people, particularly from Mexico
- Punjabi people
- Canadian First Nations
- Native Americans especially in the Southwest USA
This list may increase as we learn more, but so far it has only been observed rarely in caucasian individuals.
In the United States, ALECT2 diagnosis is most common in the southwest, likely due to the higher concentration of individuals with Hispanic ethnicity.
LECT2 amyloidosis symptoms and diagnosis
LECT2 is a naturally occurring blood protein. It is only when the protein molecules fold abnormally that the body’s ability to process LECT2 proteins is inhibited, and build up of amyloid fibers occurs.
ALECT2 is for many individuals a ‘silent’ disease, with the first indication of a problem coming from a routine blood test – much like having high cholesterol. Typical symptoms that may appear as the condition gets worse (progresses) include swelling of the legs, feet, ankles, hands or face (called edema), fatigue, and dehydration, all symptoms of kidney disease. For some individuals the progression of ALECT2 disease happens slowly, and symptoms are mild. In some, however, the damage can eventually progress to kidney failure (also known as “end stage renal disease” or ESRD), requiring dialysis or a kidney transplant.
Diagnosis of ALECT2, like all types of renal amyloidosis, requires a kidney biopsy. The initial symptoms of ALECT2 are not those that would usually prompt a doctor to request a kidney biopsy. This means that in many cases a diagnosis of ALECT 2 may be delayed until symptoms are more severe. Perhaps for this reason, many individuals are not diagnosed until middle age or later, although it has been seen in individuals as young as 30.
A physician who suspects ALECT2 would go through the following steps to arrive at a conclusive diagnosis:
Assessment of the patient’s medical and family history
Thorough physical examination
Urine and blood tests
Kidney biopsy followed by examination of the kidney tissue by amyloid experts
ALECT2 treatment options and outcomes
The recent discovery of ALECT2 means that there are no specific treatments. Current treatment options focus on supporting kidney function by monitoring and maintaining a good fluid balance. This is achieved through controlling the intake of fluid (drinks) during the course of the day, avoiding salty foods, and taking medicines called diuretics. Individuals with advanced disease (referred to as end-stage renal disease) will need dialysis. The only effective treatment currently available for ALECT2 is a kidney transplant.
Long term outcomes for people with ALECT2 are not yet clear. However in a USA study following 64 individuals with ALECT2 for an average of 2 years, it was shown that kidney function declined slowly in most cases. Approximately 1 in 3 individuals had stable kidney function, and while the rest had reduced kidney function, only approximately 1 in 3 developed end-stage renal disease.
It is important to learn as much as possible about this recently discovered amyloid condition, and research studies such as the REFLECTION natural history study are key in gathering the knowledge necessary to create new treatments for ALECT2.
What to do if you think you might have LECT2-caused kidney disease
The only way to confirm a diagnosis of ALECT2 is with a kidney (renal) biopsy, followed by examination of the kidney tissue by amyloid experts. If you think you may have LECT2 amyloidosis, but do not have a diagnosis, the following questions may help you have a productive conversation with your doctor:
To help you prepare for a discussion with your doctor, you can download our ALECT2 Doctor Information and Discussion Guide, and take it to your next appointment.