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P73 A rare case: Thrombotic thrombocytopenic purpura presenting with acute kidney injury
  1. Gamze Seval Şahin,
  2. Beltinge Demircioğlu Kılıç,
  3. Mithat Büyükçelik,
  4. Sinan Akbayram
  1. Gaziantep University, Gaziantep, Turkey


Introduction Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. Acute kidney injury (AKI) is seen very rarely in TTP. In this case report, a case of TTP presenting with severe AKI was reported.

Case A 14-year-old girl was admitted to the emergency department with the complaint of the absence of urine output for two days and side pain. On physical examination, there was no other finding except costovertebral angle sensitivity.

It was learned that she had applied to the hospital with the complaint of absence of urine output nine years ago. She had been diagnosed with atypical hemolytic uremic syndrome. ADAMTS activity could not be studied at that time. Fresh frozen plasma (FFP) infusion was applied and hemodialysis was performed three times. Then the patient was improved. However, the patient did not come for follow-up.

The laboratory findings of the patient in the latest emergency presentation were urea: 211 mg/dl, cre: 8.56 mg/dl, LDH: 5388 U/L, PLT: 103×103/µl, and coagulation values were normal. Direct coombs test was negative, and peripheral blood smear revealed schistocytes and fragmented erythrocytes.

The patient was diagnosed with TTP due to low ADAMTS 13 activity (<0.2%). Plasmapheresis and dialysis treatment was performed and also started pulse prednisolone treatment. The platelet count increased to over 150.000 on the 5th day and urine output improved on the 10th day. The patient was discharged with an oral steroid therapy.

Conclusion In this case report, we want to emphasize that severe AKI may rarely occur in TTP patients in childhood. We should evaluate the ADAMTS level of each patient with AKI and hemolytic anemia and regulate the treatment.

  • Acute kidney injury
  • ADAMTS 13
  • thrombotic thrombocytopenic purpura

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