Regarding the pathophysiology of TMA secondary to SCD, Shome et al. hypothesized that the endothelial activation that occurs during a vaso-occlusive crisis may lead to greater release of multimers of von Willebrand factor, and inhibition or reduction of the activity of ADAMTS-13 by hyperhemolysis, proteolysis and/or reduction of its synthesis, thus decreasing the cleavage of von Willebrand multimers, which leads to microangiopathic thrombosis.8 This hypothesis can be supported by the fact that these patients respond well to plasma therapy.8 The gene discussed is VWF; the disease is Schnyder corneal dystrophy.