(en) The thrombotic microangiopathy syndromes are extraordinarily diverse. Most thrombotic microangiopathy syndromes, ∼90%, occur in the setting of coexisting diseases and have been termed secondary hemolytic uremic syndrome (HUS), while a subset of thrombotic microangiopathy syndromes occur on the background of complement gene variants and are termed primary atypical HUS. 1 Le Clech et al.2 recently reported the low prevalence of such variants in patients with secondary HUS and poor clinical outcomes. [...]
Timmermans, S. A. M. E. G., Wérion, A., Morelle, J., & van Paassen, P. (2019). Defects in complement and “secondary” hemolytic uremic syndrome. Kidney International, 96(2), 517. https://doi.org/10.1016/j.kint.2019.04.011 (Original work published 2019)