Lect2 amyloidosis

LECT2 Amyloidosis, or ALect2, is one of the most recently recognized types of amyloidosis, and is also one of the most common forms, along with AL amyloidosis and AA amyloidosis. Amyloidosis results when there is abnormal deposition of protein in tissue organs, typically resulting in organ failure.

There are over 30 proteins known to cause Amyloidosis in humans. ALect2 is caused by the deposition of the Leukocyte Chemotactic Factor 2 (LECT2) protein in the kidneys, and typically cause symptoms similar to kidney failure.

LECT2 Amyloidosis presents as progressive renal failure in elderly, Hispanic patients. Associated symptoms may include fatigue, dehydration, swelling of kidneys, and blood in urine.

Some cases may also result in progressive renal insufficiency with proteinuria, as well as chronic renal insufficiency and bland urinary sediment.

A kidney biopsy is required for definitive diagnosis.

If renal amyloidosis is diagnosed, antibody or proteomic methods are used to type amyloidosis. AL amyloidosis is the most common form of amyloidosis, accounting for 85% of total cases, and can be tested by taking a blood sample. If the patient tests negative for AL, a Congo Red staining of the kidney biopsy will be done to determine if the patient has ALect2.

ALect2 is often misdiagnosed as AL amyloidosis, which is derived from immunoglobulin light chain.

End-stage renal disease is developed in 1 out of 3 patients, and the median renal survival is 62 months. A suggested prognostic tool is to track creatinine levels in ALect2 patients.

There currently is no effective treatment for LECT2 amyloidosis.

Most ALect2 patients in the United States (88%) are of Mexican descent, and are from the Southwest region of the United States (New Mexico, Arizona, far Western Texas). However, other groups with higher incidence rates include First Nation Peoples in Canada, Punjabis, Sudanese, and Native Americans.

ALect2 cases have been found in patients between 43 and 88, with a mean age of 67.

There currently is no familial link in the disease, though there have been several cases described between siblings.

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