title: [Thrombotic thrombocytopenic purpura in a newborn].
abstract: We report the case of a newborn presenting with hemolytic anemia, thrombocytopenia, hyperbilirubinemia, and renal failure in the first hours of life. An early plasmatherapy was undertaken, with good outcome. The specific von Willebrand factor-cleaving protease activity (ADAMTS 13 for a disintegrin and metalloprotease with thrombospondin type 1 repeats) was found to be low. This is the specific biologic diagnostic element of congenital thrombotic thrombocytopenic purpura (TTP). This disease of constitutional thrombotic microangiopathy is rare. The prognosis, usually life-threatening, was completely transformed given the better understanding of the pathogenesis of the disease and therapeutic progress.
authors.affiliation: Unité d'hématologie, service laboratoire de biologie clinique, CHU Mongi Slim, 2046 La Marsa, Tunisie. Electronic address: gharianiines@gmail.com.
authors.forename: I
authors.initials: I
authors.lastname: Ghariani
authors.affiliation: Laboratoire d'hématologie, CHU Farhat Hached, 4000 Sousse, Tunisie.
authors.forename: N
authors.initials: N
authors.lastname: Jmili-Braham
authors.affiliation: Service de pédiatrie, CHU Mongi Slim, 2046 La Marsa, Tunisie.
authors.forename: O
authors.initials: O
authors.lastname: Azzebi
authors.affiliation: Laboratoire d'hématologie, CHU Farhat Hached, 4000 Sousse, Tunisie.
authors.forename: M
authors.initials: M
authors.lastname: Kortas
authors.affiliation: Service d'hématologie biologique, hôpital Antoine-Béclère, 92140 Clamart, France.
authors.forename: A
authors.initials: A
authors.lastname: Veyradier
authors.affiliation: Unité d'hématologie, service laboratoire de biologie clinique, CHU Mongi Slim, 2046 La Marsa, Tunisie.
authors.forename: L
authors.initials: L
authors.lastname: Bakir
mesh_terms: D000743:Anemia, Hemolytic / Q000209:etiology; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D007231:Infant, Newborn; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis*; D051437:Renal Insufficiency / Q000209:etiology; D013921:Thrombocytopenia / Q000209:etiology