Abstract
Thrombotic microangiopathies (TMA) are a group of serious conditions that are characterized by microangiopathic hemolytic anemia and thrombocytopenia, and are often associated with acute kidney injury as well as neurologic abnormalities. There are multiple causes of TMA. TMA in a transplant patient is often attributed to calcineurin inhibitor (CNI) use and is usually treated with discontinuation of the drug. We report a case of TMA in a liver transplant patient who did not respond to CNI discontinuation or plasmapheresis but had great response to shorter than usual course of eculizumab. Eculizumab is a monoclonal antibody that prevents c5 cleavage and indirectly inhibits the formation of the membrane attack complex. Clinical response was sustained for nine months after discontinuation of eculizumab.
Highlights
Thrombotic microangiopathies (TMA) are a group of serious conditions that are characterized by microangiopathic hemolytic anemia and thrombocytopenia, and are often associated with acute kidney injury as well as neurologic abnormalities
TMA in a transplant patient is often attributed to calcineurin inhibitor (CNI) use and is usually treated with discontinuation of the drug
We report a case of TMA in a liver transplant patient who did not respond to CNI discontinuation or plasmapheresis but had great response to shorter than usual course of eculizumab
Summary
Thrombotic microangiopathies (TMA) are a group of serious conditions that are characterized by microangiopathic hemolytic anemia and thrombocytopenia, and are often associated with acute kidney injury. Ultrasound of the abdomen with Doppler showed no evidence of cirrhosis, masses and biliary dilation and demonstrated normal flow in the hepatic vessels She underwent an orthotropic liver transplant due to her acute liver failure. She was given induction chemotherapy with methylprednisolone and mycophenolate and was maintained on tacrolimus, mycophenolate and prednisone Her liver function tests (LFTs) improved to baseline post-transplant. Acute rejection of allograft liver is defined by a scoring system called the Rejection Activity Index, set by the World Gastroenterology Consensus Document It provides a score for the degree of portal, ductal and venous endothelial inflammation, lymphocyte infiltration, edema, reactive or degenerative changes and necrosis seen in the biopsy. The degree of portal inflammation, hepatocellular injury and vascular insult seen in the liver biopsy was mild and did not meet the Rejection Activity Index's score for acute rejection. On treatment she did not experience any of the commonly seen side effects of eculizumab such as headache, hypertension, diarrhea, or leukopenia or meningitis
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
