COVID-19-ASSOCIATED THROMBOTIC THROMBOCYTOPENIA PURPURA (TTP)

ABSTRACT

SESSION TITLE COVID-Related Critical Care Cases SESSION TYPE Case Reports PRESENTED ON 10/19/2022 1115 am - 1215 pm INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a rare medical emergency with mortality rates reported to be as high as 90% if untreated. We report a case of severe TTP in an immunocompetent patient diagnosed with COVID 19 infection. CASE PRESENTATION A 34-year-old morbidly obese female not vaccinated for COVID presented to PCP 2 weeks prior with complaints of fatigue. CBC showed Hb 12.2, platelet count 108 (baseline > 200), and covid resulted positive. The patient was reluctant for further workup at the time. After 2 weeks she felt short of breath prompting ICU admission for high flow oxygen with blood work showing hemoglobin of 8.8, platelet count of 11, Reticulocyte count 3.9%, LDH 763, fibrinogen 639, schistocytes on peripheral smear, MCV < 90, INR<1.5, Creatinine 1 giving her a PLASMIC Score of 7. For concerns of TTP, she was urgently started on plasmapheresis, prednisone 1 mg/kg, and remdesivir for COVID. ADAMTS13 was sent prior to the initiation of plasmapheresis resulted at 5% indicating severe deficiency and a high risk of relapse. She underwent 3 cycles of plasma exchange and was also started on Caplacizumab. Rituximab was not started in the setting of active COVID infection and negative COVID IgG. Repeat ADAMTS13 level at 2-week interval increased to 43%. The patient was discharged on steroids and completed 1 month of Caplacizumab. On outpatient follow up the patient was asymptomatic but repeat ADAMTS13 declined to 16%. COVID IgG now resulted positive, and she was started on Rituximab. Ultimately patient was treated with 4 months of steroids and 4 doses of weekly Rituximab with the final two ADAMTS 13 levels normalized above 100. DISCUSSION: TTP is caused by decreased activity of the plasma metalloproteinase ADAMTS 13, the key enzyme involved in the cleavage of ultra-large von Willebrand Factor (vWF) multimers into smaller less procoagulant multimers. It is reported that COVID-19 infection is associated with almost a five-fold increase in vWF levels which the body's ADAMTS-13 enzyme activity cannot adequately regulate, resulting in an excess of unchecked ultra-large vWF, diffuse microthrombi, and systemic ischemia. The presentation of this disease is often characterized by the pentad of fever, thrombocytopenia, hemolytic anemia, renal dysfunction, and neurologic dysfunction. However, the full pentad is often not present in many patients Plasma exchange and immunosuppression are the mainstays of treatment for TTP. A high index of suspicion is required for a timely diagnosis. Early diagnosis is crucial as without treatment TTP is associated with a high mortality rate. CONCLUSIONS: Health care providers should be aware of this life-threatening complication of COVID-19 so that prompt and appropriate interventions can be undertaken if it is suspected or confirmed. Rituximab should be delayed until the acute COVID-19 infection has cleared, and neutralizing antibodies have been produced. Reference #1 Hindilerden F, Yonal-Hindilerden I, Akar E, Kart-Yasar K. Covid-19 associated autoimmune thrombotic thrombocytopenic purpura Report of a case. Thromb Res. 2020;195136-138. doi10.1016/j.thromres.2020.07.005 DISCLOSURES No relevant relationships by Hanish Jain No relevant relationships by Dragos Manta No relevant relationships by Parth Sampat No relevant relationships by Garima Singh No relevant relationships by Simant Thapa