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Arch Pediatr. 2016 Jan;23(1):78-81. doi: 10.1016/j.arcped.2015.09.032. Epub 2015 Nov 6.

[Thrombotic thrombocytopenic purpura in a newborn].

[Article in French]

Author information

1
Unité d'hématologie, service laboratoire de biologie clinique, CHU Mongi Slim, 2046 La Marsa, Tunisie. Electronic address: gharianiines@gmail.com.
2
Laboratoire d'hématologie, CHU Farhat Hached, 4000 Sousse, Tunisie.
3
Service de pédiatrie, CHU Mongi Slim, 2046 La Marsa, Tunisie.
4
Service d'hématologie biologique, hôpital Antoine-Béclère, 92140 Clamart, France.
5
Unité d'hématologie, service laboratoire de biologie clinique, CHU Mongi Slim, 2046 La Marsa, Tunisie.

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.

PMID:
26552625
DOI:
10.1016/j.arcped.2015.09.032
[Indexed for MEDLINE]

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