Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.
Pediatr Nephrol. 2010-12-15; 26(4): 631-635
DOI: 10.1007/s00467-010-1721-9
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Thrombotic microangiopathies comprise different entities, including hemolytic
uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and several
other conditions. TTP is characterized by hemolytic anemia, thrombocytopenia, and
multiorgan failure. TTP is the result of severe von Willebrand factor multimer
cleaving protease (ADAMTS13) deficiency that is either inherited or the result of
acquired autoantibodies. We report a critically ill 2-year-old girl with invasive
pneumococcal disease associated HUS (p-HUS) whose condition was complicated by
severe ADAMTS13 deficiency, without detectable inhibitor, in a context of
multiple organ failure. The patient recovered with supportive treatment, and
ADAMTS13 activity normalized without plasmatherapy. Severe ADAMTS13 deficiency
appears to be a manifestation of transient endothelial cell injury in the context
of severe sepsis, including invasive p-HUS. The choice of appropriate therapy
should not be based on this finding.