Clinical Course and Outcomes of Thrombotic Thrombocytopenic Purpura in Young Adults with Systemic Lupus Erythematosus: A Case Series from a Tertiary Hospital

Introduction Thrombotic thrombocytopenic purpura (TTP) is a medical emergency. It remains a rare but life-threatening complication of systemic lupus erythematosus (SLE) presenting with microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, fever, and neurological symptoms. Without treatment, almost all patients die with a mortality rate of 90-100%. With plasma exchange, survival rises dramatically to nearly 90%. In lupus patients, the diagnosis is often blurred where anemia, thrombocytopenia, and neurologic symptoms can all be mistaken as lupus activity which leads to delay in management. Method We report three cases of young female who developed TTP at varying points relative to their SLE diagnoses: before, concurrently, and after. The first patient experienced relapsing TTP before ultimately developing serologic markers of SLE; she exhibited severely reduced ADAMTS13 activity with high inhibitor levels. The second patient initially presented with normal ADAMTS13 activity and high inhibitor TTP as initial presentation alongside clinical features suggestive of SLE despite negative autoimmune markers. She developed exacerbation triggered by catheter-related blood stream infection. Lastly, the third patient is a known case of SLE who presented with seizure episode attributed to TTP despite being on maintenance medications; unfortunately, the ADAMTS13 testing was not sent due to the acuteness of the event [ 1 ] [ 2 ] [ 3 ] [ 4 ] [ 5 ] [ 6 ] [ 7 ] [ 8 ] [ 9 ] [ 10 ]. Results Thrombotic thrombocytopenic purpura is most often caused by autoantibodiesagainst ADAMTS13. Determination of ADAMTS13 activity and inhibitor support and confirm its diagnosis. Severe ADAMTS13 deficiency (