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Diagnostic approach to microangiopathic hemolytic disorders
Author(s) -
KottkeMarchant K.
Publication year - 2017
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/ijlh.12671
Subject(s) - microangiopathic hemolytic anemia , thrombotic thrombocytopenic purpura , von willebrand factor , thrombotic microangiopathy , eculizumab , atypical hemolytic uremic syndrome , shiga toxin , medicine , hemolytic anemia , hemolysis , complement system , schistocyte , immunology , alternative complement pathway , complement factor b , biology , antibody , escherichia coli , platelet , biochemistry , disease , gene
Thrombotic micro‐angiopathies ( TMA ) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMA s is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura ( TTP ) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin‐related hemolytic‐uremic syndrome (Stx‐ HUS ) and complement‐mediated TMA (also called atypical hemolytic‐uremic syndrome), respectively. Distinguishing these disorders is important, as many TMA s are life‐threatening, the treatments are distinct and selecting appropriate therapy can improve patient prognosis. Laboratory testing, including measurement of ADAMTS 13, ADAMTS 13 inhibitor, shiga toxin, and complement factors, can help establish diagnoses and guide therapy.