Thrombotic microangiopathies assessment: mind the complement
Por:
Blasco, M, Guillen, E, Quintana, LF, Garcia-Herrera, A, Pineiro, G, Poch, E, Carreras, E, Campistol, JM, Diaz-Ricart, M and Palomo, M
Publicada:
1 abr 2021
Resumen:
When faced with microangiopathic haemolytic anaemia, thrombocytopenia and organ dysfunction, clinicians should suspect thrombotic microangiopathy (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 low incidence, an accurate early diagnosis is often troublesome. In the last few decades, major improvements have been made in the pathophysiological knowledge of TMAs such as thrombotic thrombocytopenic purpura [TTP, caused by ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin Type 1 motif, member 13) deficiency] and atypical haemolytic uraemic syndrome (aHUS, associated with dysregulation of the alternative complement pathway), together with enhancements in patient management due to new diagnostic 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 the management of TMA patients in the near future, reducing response times and improving patient outcomes.
Filiaciones:
Blasco, M:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Inst Biomed Res August Pi & Sunyer IDIPABS, Malalties Nefrourol & Trasplantament Renal, Barcelona, Spain
Guillen, E:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Quintana, LF:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Inst Biomed Res August Pi & Sunyer IDIPABS, Malalties Nefrourol & Trasplantament Renal, Barcelona, Spain
Garcia-Herrera, A:
Univ Barcelona, Hosp Clin, Dept Pathol, Barcelona, Spain
Pineiro, G:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Inst Biomed Res August Pi & Sunyer IDIPABS, Malalties Nefrourol & Trasplantament Renal, Barcelona, Spain
Poch, E:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Inst Biomed Res August Pi & Sunyer IDIPABS, Malalties Nefrourol & Trasplantament Renal, Barcelona, Spain
:
Hosp Clin Barcelona, Josep Carreras Leukaemia Res Inst, n Univ Barcelona Campus, Barcelona, Spain
Barcelona Endothelium Team, Barcelona, Spain
Campistol, JM:
Univ Barcelona, Hosp Clin, Ctr Referencia Enfermedad Glomerular Compleja Sis, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Inst Biomed Res August Pi & Sunyer IDIPABS, Malalties Nefrourol & Trasplantament Renal, Barcelona, Spain
Diaz-Ricart, M:
Barcelona Endothelium Team, Barcelona, Spain
Univ Barcelona, Dept Pathol, Hematopathol Unit, Hosp Clin Barcelona,Biomed Diag Ctr CDB,Inst Biom, Barcelona, Spain
:
Hosp Clin Barcelona, Josep Carreras Leukaemia Res Inst, n Univ Barcelona Campus, Barcelona, Spain
Barcelona Endothelium Team, Barcelona, Spain
Univ Barcelona, Dept Pathol, Hematopathol Unit, Hosp Clin Barcelona,Biomed Diag Ctr CDB,Inst Biom, Barcelona, Spain
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