Case 2
A previously healthy 30-year-old male presented to the emergency department with headache, fever, dysarthria and right hemiparesis. He had received a second dose of an inactivated COVID-19 vaccine CoronaVac one month prior to consultation. Laboratory findings showed hemoglobin 7.2 g/dL, platelet count, 9×109/L, LDH 1268 UI/L, D-dimer 1890µg/L, haptoglobin 0.26 g/L and creatinine 105 µmol/L. Peripheral blood smear showed schistocytes (2%). PLASMIC score was at 5 (range, 0-7). A presumptive diagnosis of TTP was made. The patient was admitted to the ICU. On the initial examination, the patient had a fluctuating consciousness, dysarthria and right hemiparesis without any petechiae or purpura. The brain CT scan revealed no abnormalities. No triggering factors such as viral infections or medication, alcohol or illicit drugs use were identified. Infectious screening tests were negative. Investigations revealed severe ADAMTS-13 deficiency (< 0.2 %) with positive anti ADAMTS-13 autoantibodies (12 U/ml). All other autoimmune tests returned negative.
The patient received methylprednisolone 1000 mg daily for three consecutive days followed by prednisone 1 mg/kg/day in combination with daily PEX. Weekly infusion of Rituximab for 4 weeks was started two weeks after admission due to issues concerning the patient’s health insurance.
The patient had a fully recovery after 31-day course including 26 sessions of PEX (figure 2 ). The patient was discharged with hemoglobin at 10 g/dl and platelets at 180 ×109/L with a follow up at the hematology department. The steroids dose was tapered off over 4 weeks. One month later, the control of activity of ADAMTS-13 was 74%.