Attending Rounds: Microangiopathic Hemolytic Anemia with Renal Insufficiency

作者：Clark William F^*; Hildebrand Ainslie

来源：Clinical Journal of the American Society of Nephrology, 2012, 7(2): 342-347.

DOI：10.2215/CJN.07230711

The classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy thrombotic thrombocytopenic purpura (TIP), hemolytic uremic syndrome (H US), and disseminated intravascular coagulation encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TIP, recognition of the absence of ADAMTS13 inhibition in diarrhea! H US, identification of complement abnormalities in atypical H US, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from H US and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy. Clin J Am Soc Nephrol 7: 342-347, 2012. doi: 10.2215/CJN.07230711

• 出版日期2012-2