Leukocyte cell-derived chemotaxin 2 (LECT2)–associated amyloidosis is a frequent cause of hepatic amyloidosis in the United States

Abstract

Key Points Leukocyte cell-derived chemotaxin-2–associated amyloidosis (ALect2) is a common cause of systemic amyloidosis involving the liver. Recognition and accurate diagnosis of hepatic ALect2 amyloidosis is essential for accurate management of patients with hepatic amyloidosis.

Leukocyte Cell–Derived Chemotaxin 2–Associated Amyloidosis

“…In sharp contrast to other types of systemic amyloidosis, ALECT2 amyloidosis exhibits a strong ethnic bias, with 88%-92% of reported patients being Hispanics (5,11,12). All reported Hispanic patients who disclosed their origin were of Mexican descent (12,16).…”

“…Of 120 patients with ALECT2 amyloidosis diagnosed by liquid chromatography/mass spectrometry (LC/MS) at the Mayo Clinic between 2007 and 2012, 72 affected the kidney, 36 liver, five spleen, three prostate, and one each from gallbladder, pancreas, small bowel, and parathyroid gland (12). Interestingly, most patients with ALECT2 amyloidosis reported so far were diagnosed by kidney biopsy in patients without clinically evident extrarenal organ involvement (5,12,17) or liver biopsy in patients without clinically evident extrahepatic organ involvement (11). A patient with biopsy-proven pulmonary ALECT2 amyloidosis was also reported (21).…”

“…Three recent large studies (5,11,12) that included a total of 144 patients (112 patients with renal amyloidosis and 32 patients with liver amyloidosis) have established that ALECT2 amyloidosis is now a frequent type of renal and hepatic amyloidosis and highlighted its distinct clinical and pathologic characteristics, including clear ethnic predisposition, typical presentation with chronic renal insufficiency with or without proteinuria, and superior patient survival to AL amyloidosis and AA amyloidosis. This mini-review highlights the current status of ALECT2 amyloidosis, including incidence, organ distribution, clinical presentation, pathogenesis, histologic characteristics, renal and patient survival, poor prognostic indicators, and recurrence in the renal allograft.…”

“…In this series, ALECT2 amyloidosis accounted for 54% of patients with renal amyloidosis (2% of total kidney biopsies received during the study period) from the southwestern United States (New Mexico, Arizona, and far west Texas) (5), which has a high concentration of Hispanics. ALECT2 amyloidosis is also a frequent cause of hepatic amyloidosis (11,15). In a recent series of 130 patients with liver amyloidosis diagnosed by proteomics at the Mayo Clinic, 62% were AL, 25% were ALECT2, 7% were AApo AI, 4% were AA, 2% were ATTR, and 1% amyloidosis derived from lysozyme (11).…”

“…ALECT2 amyloidosis is also a frequent cause of hepatic amyloidosis (11,15). In a recent series of 130 patients with liver amyloidosis diagnosed by proteomics at the Mayo Clinic, 62% were AL, 25% were ALECT2, 7% were AApo AI, 4% were AA, 2% were ATTR, and 1% amyloidosis derived from lysozyme (11).…”