Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report
Amyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by vary...
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doaj-f1ff1a9fcae146809c89a5bc295b2c782020-11-24T23:46:40ZengKarger PublishersCase Reports in Nephrology and Dialysis2296-97052017-12-017212112910.1159/000479678479678Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case ReportGagandeep KaurBabitha BijinKamron SaleemBenjamin SarsahBijin ThajudeenAmyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria. There are only a limited number of cases reported in the literature. We present a 64-year-old Hispanic female with a history of hypertension who was referred for chronic kidney disease management. The review of her laboratory tests revealed a serum creatinine of 1.5–1.8 mg/dL and microalbuminuria (in the presence of a bland urine sediment) in the past year. She denied any history of diabetes, rheumatologic disorders or exposure to intravenous contrast, nonsteroidal anti-inflammatory drugs, herbals, and heavy metals. Serological workup was negative. A renal biopsy showed diffuse infiltration of glomerulus with pale eosinophilic material strongly positive for Congo red stain and a similar eosinophilic material in the interstitium, muscular arteries, and arterioles. Electron microscopy showed marked infiltration of the mesangium, capillary loops, and interstitium with haphazardly arranged fibrillary deposits (9.8 nm thick). Liquid chromatography tandem mass spectrometry confirmed leukocyte cell-derived chemotaxin 2 (LECT2) amyloid deposition. LECT2 amyloidosis (ALECT2) should be suspected in renal biopsy specimens exhibiting extensive and strong mesangial as well as interstitial congophilia. Individuals with LECT2 renal amyloidosis have a varying prognosis. Therapeutic options include supportive measures and consideration of a kidney transplant for those with end-stage renal disease.https://www.karger.com/Article/FullText/479678ALECT2AmyloidosisChronic kidney diseaseTubulointerstitial nephritis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Gagandeep Kaur Babitha Bijin Kamron Saleem Benjamin Sarsah Bijin Thajudeen |
spellingShingle |
Gagandeep Kaur Babitha Bijin Kamron Saleem Benjamin Sarsah Bijin Thajudeen Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report Case Reports in Nephrology and Dialysis ALECT2 Amyloidosis Chronic kidney disease Tubulointerstitial nephritis |
author_facet |
Gagandeep Kaur Babitha Bijin Kamron Saleem Benjamin Sarsah Bijin Thajudeen |
author_sort |
Gagandeep Kaur |
title |
Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report |
title_short |
Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report |
title_full |
Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report |
title_fullStr |
Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report |
title_full_unstemmed |
Leukocyte Cell-Derived Chemotaxin 2-Associated Renal Amyloidosis: A Case Report |
title_sort |
leukocyte cell-derived chemotaxin 2-associated renal amyloidosis: a case report |
publisher |
Karger Publishers |
series |
Case Reports in Nephrology and Dialysis |
issn |
2296-9705 |
publishDate |
2017-12-01 |
description |
Amyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria. There are only a limited number of cases reported in the literature. We present a 64-year-old Hispanic female with a history of hypertension who was referred for chronic kidney disease management. The review of her laboratory tests revealed a serum creatinine of 1.5–1.8 mg/dL and microalbuminuria (in the presence of a bland urine sediment) in the past year. She denied any history of diabetes, rheumatologic disorders or exposure to intravenous contrast, nonsteroidal anti-inflammatory drugs, herbals, and heavy metals. Serological workup was negative. A renal biopsy showed diffuse infiltration of glomerulus with pale eosinophilic material strongly positive for Congo red stain and a similar eosinophilic material in the interstitium, muscular arteries, and arterioles. Electron microscopy showed marked infiltration of the mesangium, capillary loops, and interstitium with haphazardly arranged fibrillary deposits (9.8 nm thick). Liquid chromatography tandem mass spectrometry confirmed leukocyte cell-derived chemotaxin 2 (LECT2) amyloid deposition. LECT2 amyloidosis (ALECT2) should be suspected in renal biopsy specimens exhibiting extensive and strong mesangial as well as interstitial congophilia. Individuals with LECT2 renal amyloidosis have a varying prognosis. Therapeutic options include supportive measures and consideration of a kidney transplant for those with end-stage renal disease. |
topic |
ALECT2 Amyloidosis Chronic kidney disease Tubulointerstitial nephritis |
url |
https://www.karger.com/Article/FullText/479678 |
work_keys_str_mv |
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