TY - CHAP M1 - Book, Section TI - Thrombotic Thrombocytopenic Purpura, Hemolytic Uremic Syndrome, and Other Thrombotic Microangiopathies A1 - Zheng, X. Long A1 - Mustafa, Reem A. A1 - Mangalmurti, Nilam A2 - Schmidt, Gregory A. A2 - Kress, John P. A2 - Douglas, Ivor S. PY - 2023 T2 - Hall, Schmidt and Wood’s Principles of Critical Care, 5th Edition AB - KEY POINTSThrombotic microangiopathy (TMA) describes a syndrome of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia with various degrees of organ dysfunction, resulting from endothelial injury and disseminated microvascular thromboses.Thrombotic thrombocytopenic purpura (TTP) may be caused by hereditary or acquired (or immune-mediated) deficiency of plasma ADAMTS13 activity, while hemolytic uremic syndrome (HUS) may be caused by Shiga toxin-producing Escherichia coli (STEC) and/or inherited or acquired abnormalities in complement activation and regulation or others.TTP should be differentiated from HUS and other related TMA resulting from malignant hypertension, disseminated intravascular coagulation, collagen vascular diseases with vasculitis, catastrophic antiphospholipid syndrome, and Hemolytic anemia, Elevated Liver enzymes, and Low Platelets) (HELLP) syndrome, etc.A triple therapy, including daily therapeutic plasma exchange, caplacizumab, and immunosuppressives (e.g., corticosteroids and rituximab), is considered the standard of care for immune-mediated TTP, while intermittent plasma infusion may be sufficient for hereditary TTP.Therapy with anti-C5 monoclonal antibodies (e.g., eculizumab or ravulizumab) should be considered for atypical or complement-mediated HUS. SN - PB - McGraw Hill CY - New York, NY Y2 - 2024/10/14 UR - accesssurgery.mhmedical.com/content.aspx?aid=1201808112 ER -