Abstract

MOGOLLON MOLINA, Natalia; ROTONDO, Sabrina; DOS SANTOS, Célia  and  SANCHEZ-LUCEROS, Analía. Complement biomarkers and molecular targets in the diagnosis of thrombotic microangiopathies. Acta bioquím. clín. latinoam. [online]. 2020, vol.54, n.4, pp.437-453. ISSN 0325-2957.

The complement system plays a crucial role in the innate immune response, being the first-line defense against pathogens and regulating homeostasis. Uncontrolled complement activation can cause immunologic, hematologic as well as renal syndromes of variable severity. Among them, thrombotic microangiopathies (TMA) represent a group of rare diseases characterised by similar clinical manifestations such as microangiopathic hemolytic anemia (MAHA), peripheral thrombocytopenia and organ injury. Although TMA classification is still challenging and no international consensus has been reached, complement-associated disorders have been described thanks to the efficiency of anti-complement therapy in patients. Diagnostic tools developed in the last decades are essential to differentiate the two most well characterized TMA: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). This review will describe how the complement system works in physiological conditions in order to explain how complement abnormalities are involved in TMA, and finally how to detect those anomalies using laboratory tests.

Keywords : Thrombotic microangiopathies; Complement; Atypical hemolytic uremic syndrome; Diagnosis; Genetic variant; Biomarkers.

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