Erschienen in:
21.12.2017
Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate
verfasst von:
Hande Kizilocak, Nihal Ozdemir, Gürcan Dikme, Begum Koc, Tiraje Celkan
Erschienen in:
Journal of Thrombosis and Thrombolysis
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Ausgabe 2/2018
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Abstract
Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. Being inherited in an autosomal dominant manner, it is a medical emergency. Clinical presentations of patients may vary depending on the genetic mutations. Retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. During acute phase, fresh frozen plasma, protein C concentrates and anticoagulant therapy should be administered rapidly. Here we report a patient with homozygous protein C deficiency.