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TTP-like syndrome revealing advanced HIV infection: a case of secondary multifactorial thrombotic microangiopathy
Alessandro Marrone1,2, Martina Forestiero2,3, Leopoldo Ricioppo2
1Cellular and Molecular Cardiovascular Physiology and Pathophysiology Laboratory, Department of Biology, Ecology and Earth Sciences (DiBEST), University of Calabria, Rende, Italy.
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hematological emergency defined by severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) deficiency. However, TTP-like syndromes without ADAMTS13 reduction can occur in systemic infections such as human immunodeficiency virus (HIV), posing significant diagnostic and therapeutic challenges, particularly in high-risk and underdiagnosed populations. We report the case of a 45-year-old transgender woman who presented to the emergency department with abdominal and lumbar pain, severe anemia, and thrombocytopenia. Laboratory tests revealed schistocytes, elevated lactate dehydrogenase (LDH), and indirect hyperbilirubinemia, prompting a high PLASMIC score and the initiation of plasma exchange for suspected TTP. Subsequent investigations revealed a preserved ADAMTS13 activity and uncovered a previously undiagnosed advanced HIV infection, along with Epstein-Barr virus (EBV) and human herpesvirus 8 (HHV-8) viremia. Despite timely initiation of antiretroviral therapy and comprehensive supportive care, the patient experienced progressive clinical deterioration and died during hospitalization. This case illustrates a multifactorial secondary thrombotic microangiopathy (TMA) mimicking primary TTP, triggered by advanced HIV and possibly exacerbated by estrogen-associated prothrombotic risk. It highlights the importance of early virologic screening and broad diagnostic reasoning in high-risk patients with overlapping prothrombotic conditions, where identifying the underlying cause of secondary TMA is critical to improving outcomes and preventing missed opportunities for life-saving intervention.
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hematological emergency defined by severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) deficiency. However, TTP-like syndromes without ADAMTS13 reduction can occur in systemic infections such as human immunodeficiency virus (HIV), posing significant diagnostic and therapeutic challenges, particularly in high-risk and underdiagnosed populations. We report the case of a 45-year-old transgender woman who presented to the emergency department with abdominal and lumbar pain, severe anemia, and thrombocytopenia. Laboratory tests revealed schistocytes, elevated lactate dehydrogenase (LDH), and indirect hyperbilirubinemia, prompting a high PLASMIC score and the initiation of plasma exchange for suspected TTP. Subsequent investigations revealed a preserved ADAMTS13 activity and uncovered a previously undiagnosed advanced HIV infection, along with Epstein-Barr virus (EBV) and human herpesvirus 8 (HHV-8) viremia. Despite timely initiation of antiretroviral therapy and comprehensive supportive care, the patient experienced progressive clinical deterioration and died during hospitalization. This case illustrates a multifactorial secondary thrombotic microangiopathy (TMA) mimicking primary TTP, triggered by advanced HIV and possibly exacerbated by estrogen-associated prothrombotic risk. It highlights the importance of early virologic screening and broad diagnostic reasoning in high-risk patients with overlapping prothrombotic conditions, where identifying the underlying cause of secondary TMA is critical to improving outcomes and preventing missed opportunities for life-saving intervention.
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