Leukocyte Cell–Derived Chemotaxin 2–Associated Amyloidosis

Abstract

Amyloidosis derived from leukocyte cell

De novo leukocyte chemotactic factor 2 amyloidosis in a pediatric renal allograft, 15 years post‐transplant

“…Its expression is upregulated by activation of the Wnt/β‐catenin signaling pathway in the liver, a commonly dysregulated pathway in carcinogenesis . LECT2 has been demonstrated to play a role in chemotaxis, cell proliferation, immunomodulation, damage/repair processes, and glucose metabolism . However, the precise role of LECT2 in renal diseases remains unclear, and the etiology of ALECT2 is unknown.…”

“…It has been proposed that LECT2 overexpression by hepatocytes, either constitutively due to genetic factors or compensatory to hepatocellular damage, results in production of misfolded LECT2 protein. In turn, other factors, such as increased local concentration, interactions with the extracellular matrix, and binding to serum amyloid P, chaperones such as apoE, and glycosaminoglycans may lead to stabilization of misfolded LECT2 …”

“…About one‐quarter to one‐third of patients have concurrent kidney disease, most commonly diabetic nephropathy or IgA nephropathy . ALECT2 deposits preferentially in the cortical interstitium, in contrast to AL and AA amyloidosis, which typically involve the glomeruli and vessels . Liver involvement (median age 60.5 years) is typically an incidental finding discovered in liver specimens sampled for other conditions .…”

“…Amyloid proteins have a characteristic beta‐pleated sheet conformation and can be identified histologically by apple‐green birefringence when stained with Congo red and examined under polarized light. Thirty‐one amyloid precursor proteins have been described, with Ig light chain‐derived AL the most frequent type in the United States and Europe and serum AA the most frequent in developing countries . The kidney is the most common organ involved in systemic amyloidosis, and the overall incidence in renal biopsies is 1.3%‐4% .…”

“…Thirty‐one amyloid precursor proteins have been described, with Ig light chain‐derived AL the most frequent type in the United States and Europe and serum AA the most frequent in developing countries . The kidney is the most common organ involved in systemic amyloidosis, and the overall incidence in renal biopsies is 1.3%‐4% . In 2008, a new form of amyloidosis derived from LECT2 was described in a 61‐year‐old patient with a 7‐year history of nephrotic syndrome and renal insufficiency who underwent nephrectomy for clear cell renal cell carcinoma .…”