A CASE REPORT: EXTREMELY RAPID AND FATAL PROGRESSION OF NEPHROTIC SYNDROME AND LIVER FAILURE IN A PATIENT WITH IMMUNOGLOBULIN LIGHT CHAIN AMYLOIDOSIS

 

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A CASE REPORT: EXTREMELY RAPID AND FATAL PROGRESSION OF NEPHROTIC SYNDROME AND LIVER FAILURE IN A PATIENT WITH IMMUNOGLOBULIN LIGHT CHAIN AMYLOIDOSIS

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Keiichiro
Akino
Keiichiro Akino keia2000@icloud.com Keiyu Hospital Nephrology Yokohama Japan *
Kentarto Fujii kfujii225@gmail.com Keiyu Hospital Nephrology Yokohama Japan -
Tatsuya Itoh idtasy.0346@gmail.com Keiyu Hospital Nephrology Yokohama Japan -
Yoshikazu Hara y-hara@keiyu-hospital.com Keiyu Hospital Nephrology Yokohama Japan -
Hiroto Matsuda acehiro2011@yahoo.co.jp Keiyu Hospital Nephrology Yokohama Japan -
Yoshiaki Fujii yfujii@coffee.ocn.ne.jp Keiyu Hospital Nephrology Yokohama Japan -
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 Immunoglobulin light chain (AL) amyloidosis is characterized by systemic deposition of amyloid fibrils in multiple organs. Organ dysfunction due to amyloid deposition is a major cause of mortality and morbidity. The prognosis of AL amyloidosis is poor, especially in patients with cardiac involvement, with a median survival duration of approximately 6 months. 

Case report

A 59-year-old man was hospitalized due to progressive edema and body weight gain of 7 kg within 2 weeks. The patient was diagnosed with nephrotic syndrome with proteinuria (3.39 g/day and hypoalbuminemia (serum albumin 1.8 g/dL). Although 60 mg of oral prednisolone was initiated, proteinuria and edema did not improve. A rapid decline in urine output was observed, and hemodialysis was initiated on day 16. Although urinary Bence Jones protein-κ was detected, no monoclonal immunoglobulin was identified in the serum. Bone marrow biopsy revealed no increase in plasma cells; however, renal biopsy demonstrated Congo Red–positive deposits in the glomeruli, leading to a diagnosis of AL-κ amyloidosis. As hemodialysis became challenging due to hypotension, continuous hemodiafiltration was initiated on day 20. From day 24 onward, marked elevations in hepatobiliary enzyme and amylase levels were observed. Plasma exchange was ineffective, and the patient died on hospital day 32. Autopsies confirmed extensive amyloid deposition in multiple organs, including the heart, liver, kidneys, and pancreas. 

In the present case, cardiac function was preserved, and lethal arrhythmia was not observed despite autopsy confirming cardiac amyloidosis. In this patient, acute circulatory failure appeared to result from decreased effective circulating plasma volume secondary to enhanced vascular permeability. Moreover, concomitant hepatic failure and pancreatitis further exacerbated vascular permeability, ultimately leading to circulatory collapse. In patients with AL amyloidosis, a strong propensity for tissue deposition can lead to a rapid and fatal outcome due to acute circulatory failure, even in the absence of clinically apparent cardiac involvement.

Kewords