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Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome

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Abstract

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.

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Abbreviations

HUS:

hemolytic uremic syndrome

p-HUS:

pneumococcal hemolytic uremic syndrome

STEC:

Shiga-toxin-producing Escherichia coli

TF antigen:

Thomsen-Friedenreich antigen

TTP:

thrombotic thrombocytopenic purpura

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Acknowledgements

We thank Pr. Agnes Veyradier and her collaborators, Hôpital Antoine Béclère, Paris, for carrying out the confirmatory ADAMTS13 activity measurements.

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Authors and Affiliations

  1. Service de Pédiatrie, Hôpital Pellegrin-Enfants, Centre Hospitalier Universitaire, Bordeaux, France

    Sybille Pelras, Frédéric Villega, Brigitte Llanas & Jérôme Harambat

  2. Service de Néphrologie, Hôpital Pellegrin, Centre Hospitalier Universitaire, Bordeaux, France

    Yahsou Delmas

  3. Centre de référence Maladies Rénales Rares du Sud-Ouest (Sorare), Bordeaux, France

    Yahsou Delmas, Brigitte Llanas & Jérôme Harambat

  4. Service de Réanimation Pédiatrique, Hôpital Pellegrin-Enfants, Centre Hospitalier Universitaire, Bordeaux, France

    Delphine Lamireau, Paul Nolent & Olivier Brissaud

  5. Laboratoire d’Hématologie, Hôpital Pellegrin, Centre Hospitalier Universitaire, Bordeaux, France

    Anne Ryman

  6. Hôpital Pellegrin-Enfants, place Amélie Raba Léon, 33076, Bordeaux cedex, France

    Jérôme Harambat

Authors
  1. Sybille Pelras
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  2. Yahsou Delmas
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  3. Delphine Lamireau
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  4. Frédéric Villega
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  6. Anne Ryman
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  7. Brigitte Llanas
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  8. Olivier Brissaud
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  9. Jérôme Harambat
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Correspondence to Jérôme Harambat.

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Pelras, S., Delmas, Y., Lamireau, D. et al. Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome. Pediatr Nephrol 26, 631–635 (2011). https://doi.org/10.1007/s00467-010-1721-9

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  • DOI: https://doi.org/10.1007/s00467-010-1721-9

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