Thrombotic microangiopathies assessment : mind the complement
Blasco Pelicano, Miquel (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Guillén, Elena (Hospital Clínic i Provincial de Barcelona)
Quintana, Luis F. (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Garcia-Herrera, Adriana (Hospital Clínic i Provincial de Barcelona)
Piñeiro, Gastón (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Poch, Esteban (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Carreras, Enric (Institut Germans Trias i Pujol. Institut de Recerca contra la Leucèmia Josep Carreras)
Campistol Plana, Josep M (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Diaz-Ricart, Maribel (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Palomo, Marta (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Universitat Autònoma de Barcelona

Data:	2021
Resum:	When faced withmicroangiopathic haemolytic anaemia, thrombocytopenia and organ dysfunction, clinicians should suspect thromboticmicroangiopathy (TMA). The endothelial damage that leads to this histological lesion can be triggered by several conditions or diseases, hindering an early diagnosis and aetiological treatment. However, due to systemic involvement in TMA and its lowincidence, an accurate early diagnosis is often troublesome. In the last few decades,major improvements have beenmade in the pathophysiological knowledge of TMAs such as thrombotic thrombocytopenic purpura [TTP, caused by ADAMTS-13 (a disintegrin andmetalloproteinase with a thrombospondin Type 1motif,member 13) deficiency] and atypical haemolytic uraemic syndrome (aHUS, associated with dysregulation of the alternative complement pathway), together with enhancements in patientmanagement due to newdiagnostic tools and treatments. However, diagnosis of aHUS requires the exclusion of all the other entities that can cause TMA, delaying the introduction of terminal complement blockers, which have shown high efficacy in haemolysis control and especially in avoiding organ damage if used early. Importantly, there is increasing evidence that other forms of TMA could present overactivation of the complement system, worsening their clinical progression. This review addresses the diagnostic and therapeutic approach when there is clinical suspicion of TMA, emphasizing complement evaluation as a potential tool for the inclusive diagnosis of aHUS, as well as for the improvement of current knowledge of its pathophysiological involvement in other TMAs. The development of both new complement activation biomarkers and inhibitory treatments will probably improve themanagement of TMA patients in the near future, reducing response times and improving patient outcomes.
Ajuts:	Agència de Gestió d'Ajuts Universitaris i de Recerca 017-SGR675
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Publicat a:	Clinical Kidney Journal, Vol. 14 Núm. 4 (january 2021) , p. 1055-1066, ISSN 2048-8513

DOI: 10.1093/ckj/sfaa195
PMID: 33841853

12 p, 1.3 MB

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Documents de recerca > Documents dels grups de recerca de la UAB > Centres i grups de recerca (producció científica) > Ciències de la salut i biociències > Institut d'Investigació en Ciencies de la Salut Germans Trias i Pujol (IGTP) > Institut de Recerca contra la Leucèmia Josep Carreras
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