Abstract

INTRODUCTION: Thrombotic microangiopathy (TMA), which may occur in relation to hematopoietic stem cell transplant (SCT) or solid-organ transplant, refers to inflammatory and thrombotic diseases of the microvasculature characterized by otherwise unexplained microangiopathic hemolytic anemia and thrombocytopenia.We aimed to present a liver transplant recepient with TMA developed after liver transplantation. 

CASE: A 54 years old woman with advanced decompansated liver disease referred to our hospital for liver transplantation. She had a variceal bleeding history with grade III esophageal varices and tense ascites. İnitial laboratory tests revealed: Ast:38u/l, Alt:40u/l,Alp:344u/l,Ggt:68u/L, T.Bilirübin:16,11mg/dl,Direct Bilirubin:13,75mg/dl, Albumin:2,6 G/dl,Creatine.0,6mg/Dl,Hemogobin:12.1 g/dl,Hematocrites:%36.5, White blood cell count:7390,Platelets:143000,Prothrombin time:24,4,Inr:1,90. MELD score was 24, Child Pugh Hugh Score was 11 ( stage C ). Living donor liver transplantation was performed. After liver transplantation pancitopenia developed which did not resolved with blood transfusion and thrombocyte replacement. Hemotology specialist offerred perferic blood smear. In peripheral blood smear, widespread schistocytes and hemochromatosis were found to be compatible with hemolysis, the corrected reticulocyte count was detected at the upper limit (1.96%). Bone marrow aspiration revealed:increase in erythroid mass without infiltration. ADAMTS-13 activity was detected %40.5 (references 40-130). Hematology specialist’s diagnose was TA-TMA due to immunsupresive drug (Tacroliumus). Tacrolimus was stopped and we continued immunsupresion with 1mg/kg methyl prednisolone. Plasmapheresis was initialized. Treatment after 2 weeks platelet number was 27000. We have applied for the use of Eculizumab to the Ministry of Health.

CONCLUSION: Transplant-associated TMA (TA-TMA) is a rare and life threatening complication of liver transplantation. Pathogenesis is multifactorial and risk factors include exposure to calcineurin inhibitors (CNIs), unrelated donor, human leukocyte antigen mismatch, graft-versus-host disease (GVHD), and viral infections. The treatment options are not clearly defined and suboptimal with high associated mortality rates. Multidisciplinary aprroach is essential for rapid diagnose and treatment. Ecluzimab may be given in patient who does not response supportive treatment such as plazmepheresis and steroids.