Homozygous Protein C Deficiency Presenting As Neonatal Purpura Fulminans: Management with Fresh Frozen Plasma, Low Molecular Weight Heparin and Protein C Concentrate

Overview

Journal J Thromb Thrombolysis

Specialty Cardiology & Vascular Diseases

Date 2017 Dec 23

PMID 29270858

Citations 3

Authors

Hande Kizilocak

Nihal Ozdemir

Gurcan Dikme

Begum Koc

Tiraje Celkan

Affiliations

Soon will be listed here.

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.

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