Atypical presentation of atypical amyloid

Department of Pathology University of Pittsburgh Medical Center-Montefiore, Pittsburgh, PA, USA.
Nephrology Dialysis Transplantation (Impact Factor: 3.58). 10/2010; 26(1):373-6. DOI: 10.1093/ndt/gfq638
Source: PubMed


Amyloidosis is a group of diseases categorized by precipitation of a group of protein aggregates (amyloid) in tissues, including
the kidney, and proteinuria is usually the commonest, though not exclusive, hallmark of clinical presentation. AL and AA are
the most commonly recognized forms of amyloidosis involving the kidney, but other forms have been described. We present a
case of renal amyloidosis due to a novel amyloidogenic protein, leucocyte cell-derived chemotaxin 2, without proteinuria at
presentation or on subsequent follow-up.

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Available from: Danniele Holanda, Oct 08, 2015
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    ABSTRACT: Leukocyte chemotactic factor-2 (LECT2) amyloidosis has been described as being associated with kidney disease; however, no clinical manifestations outside of the kidney have been previously reported. We describe a patient presenting with pulmonary-renal syndrome found to have deposition of amyloidogenic LECT2 (ALECT2) within both the lung and the kidney. This case is unique in regard to both the patient's clinical presentation of pulmonary-renal syndrome in the setting of amyloidosis and the biopsy finding of ALECT2 deposition within the lung. It also emphasizes the importance of tissue diagnosis in such cases, given that amyloidosis was not initially considered in the differential diagnosis.
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