2022 Volume 63 Issue 11 Pages 473-481
A 70-year-old male with alcohol-related liver cirrhosis was admitted with alcoholic hepatitis. Laboratory data indicated nephrotic syndrome with hematuria. Proteinuria was resolved after alcohol abstinence and treatment with diuretics and nutrition therapy. A renal biopsy performed for hematuria demonstrated thrombotic microangiopathy (TMA) showing numerous deposits and endothelial cell dysfunction. The patient was diagnosed with TMA associated with alcoholic liver disease, along with a decreased ADAMTS13 activity and an increased serum level of von Willebrand factor (VWF). Fresh frozen plasma was transfused for the supplementation of ADAMTS13, and recombinant thrombomodulin products (rTM) were administered for thrombolysis. Cases of hematuria with a decreased ADAMTS13 activity or an imbalance in VWF warrant suspicion of TMA, and FFP/rTM should be considered in patients with cirrhosis.