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Journal de Pharmacie Clinique

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Treatment of an Upshaw-Schulman syndrome in a preterm neonate Volume 31, issue 2, Juin 2012

Authors
Service de pharmacie, Hôpital Jean Verdier - APHP, Hôpitaux Universitaires Paris Seine-Saint-Denis, Bondy, Service de néonatalogie, Hôpital Jean Verdier - APHP, Hôpitaux Universitaires Paris Seine-Saint-Denis, Bondy, Service d’hématologie, Hôpital Jean Verdier - APHP, Hôpitaux Universitaires Paris Seine-Saint-Denis, Bondy

We present the initial therapeutic management of a congenital thrombotic thrombocytopenic purpura (CTTP) in a premature (34 weeks) and diagnosed in the neonatal period. The association of hemolytic anemia and thrombocytopenia resisting to the conventional and symptomatic treatment as platelet transfusion and immunoglobulin infusion leads to the diagnosis of CTTP. After confirmation of diagnosis (significant decrease <5% of ADAMTS13 activity), it is decided to set up infusions of fresh frozen plasma (FFP). The efficiency and stabilization of hematological parameters are obtained with infusions of FFP every week. We discuss in this section of road to the diagnosis of an Upshaw-Schulman syndrome and the establishment of an alternative treatment to conventional treatment.