Abstract

Objective: In this case report ,we describe our experience with Tacrolimus induced TTP in pediatric LDLT and early use of Everolimus as immuosupression. Method: Eight year old male child with DCLD due to cholesterol ester storage disorder Underwent LDLT .POD5 there was raised in bilirubin and thrombocytopenia(2.2 lakh to 20,000 /uL) ,elevated LDH (1660U/L ) and PS showed schistocytes (5-6%) F/S/O TTP .Patient developed seizures .MRI brain showed F/S/O PRESS. Tacrolimus was withheld .Patient was started on Retuximab therapy and he underwent 5 cycles of plasmapheresis. Gradually LFT's and Blood pictures normalized and patient was discharged on POD20. Thirteen year old male child presenting with cryptogenic cirrhosis underwent LDLT. POD 10 he developed seizures and hematuria. NCCT brain showed water shed area with infarcts. POD16 patient had elevated bilirubin , Thrombocytopenia ,PS showed Schistocytes .Patient underwent 3 cycles of plamapheresis.LDH and Schistocytes levels reduced .However we lost the child to sepsis on POD 30. Results: Diagnostic criteria for TTP include Severe thrombocytopenia30 X 10 9/L, Elevated LDH, Microangiopathic Hemolytic anemia,Schistocytes on the blood smear. Clinical presentation includes Brain 60% stroke, Heart ischemia 25%, Mesenteric ischemia 35% and Hematuria. Treatment includes plasmaphersis, steroids and Rituximab. Organ transplantation associated TTP is not the result of an immune-mediated ADAMTS13 deficiency and has the Worst prognosis. Conclusion: Very few cases of post LDLT with TTP has been mentioned in literature review. To best of our knowledge this is the only case in which Everolimus has been used as immunosuppressant early post op period in TTP.

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