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Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate

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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.

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Fig. 1
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Abbreviations

FFP:

Fresh frozen plasma

LMWH:

Low molecular weight heparin

NICU:

Neonatal intensive care unit

NPF:

Neonatal purpura fulminans

PC:

Protein C

VTE:

Venous thromboembolism

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Authors and Affiliations

  1. Division of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Hande Kizilocak, Gürcan Dikme, Begum Koc & Tiraje Celkan

  2. Division of Pediatric Hematology-Oncology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey

    Nihal Ozdemir

Authors
  1. Hande Kizilocak
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  2. Nihal Ozdemir
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  3. Gürcan Dikme
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  4. Begum Koc
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  5. Tiraje Celkan
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Correspondence to Hande Kizilocak.

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The authors declare that they have no conflict of interest.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Informed consent was obtained from all individual participants included in the study.

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Kizilocak, H., Ozdemir, N., Dikme, G. et al. Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate. J Thromb Thrombolysis 45, 315–318 (2018). https://doi.org/10.1007/s11239-017-1606-x

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  • DOI: https://doi.org/10.1007/s11239-017-1606-x

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