Abstract: SA-PO381
A 64-Year-Old Woman with Raccoon Eyes After Kidney Biopsy
Session Information
- Genetic and Diagnostic Trainee Case Reports
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 2101 CKD (Non-Dialysis): Epidemiology, Risk Factors, and Prevention
Authors
- Mei, Shuqin, Shanghai Changzheng Hospital, Shanghai, China
- Mei, Changlin, Shanghai Changzheng Hospital, Shanghai, China
- Dai, Bing, Shanghai Changzheng Hospital, Shanghai, China
Introduction
Raccoon eyes is caused by blood tracking into periorbital tissues, which are easliy recognized as a symptom of basal skull fractures. However, it may be a sign of health threatening situations such as multiple myeloma, amyloidosis and so on. Here we discuss a patient with raccoon eyes after the kidney biospy who was finally diagnosed as immunoglobulin light chain (AL) amyloidosis.
Case Description
A 64-year old woman presented to our clinic with 1-year proteinuria. Laboratory study showed Scr was 382 umol/L and 24-hour urine protein quantification was 2.4 g. The testing for monoclonal protein by serum revealed an M-peak in the λ fraction of IgA (Fig1a). The concentrations of κ and λ were 44.95 and 173 mg/L, respectively. The bone marrow cytology test was negative. Ultrasound report indicated the size of right kidney was 9.1×4.4 cm and the left one was normal. But unexpectedly, the patient showed periorbital purpura 24 hours later after kidney biopsy (Fig1b). Congo red staining was positive and also showed strongly λ deposition. EM showing expansion of the mesangium by amyloid fibrils.
Discussion
AL amyloidosis is the most common type of systemic amyloidosis. Renal involvement accounts for almost 70% and most presents as clinically apparent nephrotic syndrome. Sometimes it only presented with proteinuria and slowly progressive deterioration of renal function. So it is necessary to perform tissue biospy once the patients with unknown renal failure accompany with monoclonal M protein.
The vascular infiltration of amyloid fibrils in blood vessels in patients with amyloidosis can cause bilateral periorbital ecchymosis by a Valsalva maneuver or minor trauma. Here we firstly reported this rare symptom after kidney biospy in a patient who was finally diagnosed as AL amyloidosis. During the kidney biopsy, the patient was asked to hold breath after inhalation, which mimicked Valsalva maneuver, and thus contribute to the periorbital purpura. Therefore, periorbital ecchymosis warrant more attention as an early cue of amyloidosis.
Fig1 (a) M-peak in the λ of IgA. (b) The patient showed bilateral periorbital ecchymosis.