Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Cancer Patients
Two thrombotic microangiopathies that may be managed in an intensive care unit setting include thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Although these two disorders may have similar characteristics, the underlying pathophysiology, workup, management, and prognosis are very different. Clinicians should consider these disorders when patients are admitted with microangiopathic hemolytic anemia and thrombocytopenia.
Thrombotic thrombocytopenic purpura, most commonly arises from development of an autoantibody to the metalloproteinase ADAMTS13 enzyme, is considered a medical emergency and warrants emergent initiation of treatment, typically with therapeutic plasma exchange, even prior to confirmation of the diagnosis. Hemolytic uremic syndrome is now separated into two distinct categories: primary versus secondary. Primary HUS, formerly diarrhea-negative or “atypical” HUS, is now referred to as complement-mediated HUS and is caused by a complement factor autoantibody or either loss- or gain-of-function mutations that dysregulate self-preservation against complement-induced membrane attack complex. Secondary HUS, formerly referred to as diarrhea-positive HUS, is caused by shiga toxin-induced damage to endothelial cells, renal cells, and epithelial cells resulting in a proinflammatory and prothrombotic state.
KeywordsThrombotic thrombocytopenic purpura TTP Hemolytic uremic syndrome HUS Atypical hemolytic uremic syndrome Complement-mediated HUS
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