Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso

Abstract

Haemolytic uraemic syndrome (HUS) is a clinical entity defined as the triad of nonimmune haemolytic anaemia, thrombocytopenia, and acute renal failure, in which the underlying lesions are mediated by systemic thrombotic microangiopathy (TMA). Different causes can induce the TMA process that characterizes HUS. In this document we consider atypical HUS (aHUS) a sub-type of HUS in which the TMA phenomena are the consequence of the endotelial damage in the microvasculature of the kidneys and other organs due to a disregulation of the activity of the complement system. In recent years, a variety of aHUs-related mutations have been identified in genes of the the complement system, which can explain approximately 60% of the aHUS cases, and a number of mutations and polymorphisms have been functionally characterized. These findings have stablished that aHUS is a consequence of the insufficient regulation of the activiation of the complement on cell surfaces, leading to endotelial damage mediated by C5 and the complement terminal pathway. Eculizumab is a monoclonal antibody that inhibits the activation of C5 and blocks the generation of the pro-inflammatory molecule C5a and the formation of the cell membrane attack complex. In prospective studies in patients with aHUS, the use of Eculizumab has shown a fast and sustained interruption of the TMA process and it has been associated with significative long-term improvements in renal function, the interruption of plasma therapy and important reductions in the need of dialysis. According to the existing literature and the accumulated clinical experience, the Spanish aHUS Group published a consensus document with recommendations for the treatment of aHUs (Nefrologia 2013;33[1]:27-45). In the current online version of this document, we update the aetiological classification of TMAs, the pathophysiology of aHUS, its differential diagnosis and its therapeutic management.

Subcapsular liver hematoma as a complication of an atypical hemolytic uremic syndrome

“…1 The decision to start eculizumab in our patient was made one week after presentation but the high cost of Eculizumab limited its immediate availability and forced us to rely on extensive and ineffective PEX while waiting for the immunoglobulin. Plasma exchange consisted of one volume exchange with fresh frozen plasma.…”

“…1 Extra renal manifestations are observed in 20% of patients. 2 A 42-year-old woman with unremarkable past medical history presented in our hospital reporting a 6-day history of headache, nausea and vomiting.…”

“…Discussion: Complement system dysregulation in aHUS induces thickening and inflammation of vascular wall, widening of subendothelial space and microvascular thrombosis. 1 Renal microvasculature is predominantly affected but extrarenal ischemic events, mainly involving central nervous system, were also reported. 2 Few hemorrhagic events were also reported in patients with infection-induced HUS and in secondary causes of ATM.…”

Thrombotic microangiophatic in the ICU, hemolytic uremic syndrome relate to pregnancy: case report and literature review

“…En los EE.UU., el SHUa se estima que tiene una tasa de incidencia anual de 1-2 casos por millón de habitantes 5 y, en Europa, un estudio internacional multicéntrico informó una incidencia de 0,11 casos por millón de habitantes entre las edades de 0 y 18 años 6,9 . La distribución hombre: mujer es equitativa en la infancia, pero tiende a predominar en las mujeres en la vida adulta Las manifestaciones clínicas pueden ser múltiples y muy variadas en cada paciente.…”

Síndrome urémico hemolítico atípico asociado a neumococo

“…La lesión anatomopatológica que le caracteriza consiste en un estrechamiento de las paredes arteriolares y capilares producida por la presencia de células endoteliales prominentes y edematosas con acumulación subendotelial de proteínas y la presencia de trombos plaquetarios con obstrucción de la luz vascular (2,3) . El paso de los glóbulos rojos a través de la pared vascular alterada produce la anemia hemolítica, mientras la trombocitopenia se produce por adhesión plaquetaria al endotelio dañado y la lesión renal es ocasionada por microangiopatía (1,2,4) .…”