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<p>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.</p>

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TTP-like syndrome revealing advanced HIV infection: a case of secondary multifactorial thrombotic microangiopathy

  • 期刊论文

  • 发表日期：

    2026年7月1日

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  • Alessandro Marrone,
  • Martina Forestiero,
  • Leopoldo Ricioppo,
  • Elvira Stamile,
  • Livia Bernardi,
  • Francesca Sinopoli,
  • Antonio Mastroianni,
  • Francesca Greco,
  • Nadia Caruso,
  • Maria Raffaella Ambrosio,
  • Tommaso Angelone,
  • Rocco Malivindi,
  • Cinzia Giordano,
  • Stefania Catalano

摘要

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.