A Chimeric Case of AKI
October 22, 2020 | 10:00 AM - 12:00 PM
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A Chimeric Case of AKI
- Pathology and Lab Medicine: Clinical
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1602 Pathology and Lab Medicine: Clinical
- Kharadjian, Talar, University of California San Diego, La Jolla, California, United States
- Miracle, Cynthia, University of California San Diego, La Jolla, California, United States
- Zhang, Haiyan, University of California San Diego, La Jolla, California, United States
New-onset nephrotic syndrome often requires kidney biopsy for diagnosis. Pathology is at times insufficient.
An 81-year-old Hispanic female with HTN, CKD stage III presented with rash, fever, peripheral eosinophilia, AKI and diagnosed with DRESS syndrome due to allopurinol started 3 weeks ago. Urinalysis showed pyuria, hematuria. Spot urine Pr/Cr was 3.8 with serum albumin 2.5g/dL. Admission creatinine was 4.3mg/dL, increased to 6.5mg/dL prior to starting dialysis. Serologic findings shown below.
Steroids were started for DRESS and suspected AIN. Genetic testing for HLA-B*5801, seen in severe cutaneous adverse reactions to allopurinol, was positive. Skeletal survey demonstrated no lesions. Blood counts revealed anemia and thrombocytopenia.
Bone marrow biopsy did not show a plasma cell dyscrasia. Kidney biopsy noted amyloidosis (Congo red positive deposits in glomeruli, vessels, interstitium) with 56% global glomerulosclerosis and >60% severe IFTA. Mild interstitial inflammation with occasional eosinophils suggested resolving AIN. Severe findings of hypertensive sequelae noted. Immunofluorescence was 2+ for IgG and kappa. Electron microscopy showed 10nm fibrils and severe podocyte foot process effacement. Mass spectrometry diagnosed ALECT2 amyloidosis.
Our case is unusual in having three simultaneous pathologies, namely AIN and ALECT2 amyloidosis superimposed on a background of hypertensive changes; the recognition of disproportionate proteinuria was essential to question the diagnosis and avoid early closure on AIN. Concurrent renal pathologies are a common finding in patients with ALECT2 amyloidosis, underscoring the utility of kidney biopsy in these patients who often have mixed clinical pictures.
Circumstances leading to this case raise suspicion for preceding immune dysregulation, in the form of DRESS/steroids, that may have triggered amyloidogenesis. A prior case report describes a patient who developed ALECT2 amyloid after steroids for allergic symptoms. This potential interplay between genetic and environmental factors requires further investigation.
|UPEP, urine immunofixation||No monoclonal protein|
|SPEP, serum immunofixation||Polyclonal|
|Kappa/lambda light chains||8.42|
|Beta-2 microglobulin||20.2 mg/dL|
|ANA, ANCA, anti-GBM||negative|
|Hepatitis B and C, cryoglobulin||negative|