title: Syndromes of Thrombotic Microangiopathy.
abstract: Thrombotic thrombocytopenia purpura (TTP) and the hemolytic uremic syndrome (HUS) are rare thrombotic microangiopathies that can be rapidly fatal. Although the acquired versions of TTP and HUS are generally highest on this broad differential, multiple rarer entities can produce a clinical picture similar to TTP/HUS, including microangiopathic hemolysis, renal failure, and neurologic compromise. More recent analysis has discovered a host of genetic factors that can produce microangiopathic hemolytic syndromes. This article discusses the current understanding of thrombotic microangiopathy and outlines the pathophysiology and causative agents associated with each distinct syndrome as well as the most accepted treatments.
authors.affiliation: Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA.
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Shatzel
authors.affiliation: Division of Hematology and Medical Oncology, The Hemophilia Center, Portland VA Medical Center, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, L586, Portland, OR 97239, USA. Electronic address: taylojas@ohsu.edu.
authors.forename: Jason A
authors.initials: JA
authors.lastname: Taylor
mesh_terms: D000071120:ADAMTS13 Protein / Q000378:metabolism; D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D006463:Hemolytic-Uremic Syndrome / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000503:physiopathology* / Q000628:therapy; D057049:Thrombotic Microangiopathies / Q000209:etiology / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy
chemical_list: D000276:Adjuvants, Immunologic; D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; C481642:eculizumab; D000071120:ADAMTS13 Protein
keywords: Atypical hemolytic uremic syndrome (aHUS); Hemolytic uremic syndrome (HUS); Microangiopathic hemolytic anemia (MAHA); Pregnancy induced microangiopathic hemolytic anemia; Thrombotic thrombocytopenic purpura (TTP); Transplant induced microangiopathic hemolytic anemia