Rare manifestation of purpura fulminans in newborn: Case study

Czech version

Authors: D. Chovancová ¹; I. Hartmannová ¹; Ľ. Pejhovská ¹; H. Švarcová ¹; M. Grešíková ²
Authors‘ workplace: Novorodenecká klinika M. Rusnáka LF SZU a Univerzitná nemocnica, Bratislava ¹; Pracovisko hematológie a transfúziológie oddelenia laboratórnej medicíny, Národný ústav detských chorôb, Bratislava ²
Published in: Čes-slov Neonat 2024; 30 (2): 129-135.
Category: Case Reports

Overview

Rare manifestation of purpura fulminans in newborn: Case study

Purpura fulminans is a highly thrombotic subtype of disseminated intravascular coagulation that can accompany severe bacterial, and more rarely, viral infections. It is associated with an extremely high mortality rate and patients often die of overwhelming multisystemic thrombosis rather than septic shock. Theoretical part is dealing with mechanisms underlying the dysregulation of coagulation, therapy and outcome. Management involves treatment of the underlying infection, aggressive anticoagulation, and robust transfusion support aimed at correcting acquired deficiencies in natural anticoagulant proteins. Purpura fulminans may also be the presenting symptom of severe heritable deficiency of the natural anticoagulants protein C or protein S. In the part of case study the presentation of clinical course, laboratory parameters and treatment of a neonate is discussed. Considering the rapid evolution of the pathogenetic mechanism, early identification, and prompt intervention are imperative for improved outcomes. The majority of purpura fulminant cases have an infectious etiology, but it is essential to consider other congenital and acquired causes.

Keywords:

neonate – disseminated intravascular coagulation – purpura fulminans – protein C – protein S

Sources

Adcock DM, Brozna J, Marlar RA. Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990; 16(4): 333−40. doi: 10.1055/s-2007- 1002686. PMID: 2281322. Dostupné z https://pubmed.ncbi.nlm. nih.gov/2281322.
Bektas F, Soyuncu S. Idiopathic purpura fulminans. Am J Emerg Med 2011; 29(4): 475.e5−e6. doi: 10.1016/j.ajem.2010.04.022. Epub 2010 Aug 2. PMID: 20674230. Dostupné z https://pubmed. ncbi.nlm.nih.gov/20674230.
Colling ME, Bendapudi PK. Purpura fulminans: mechanism and management of dysregulated hemostasis. Transfus Med Rev 2018; 32(2): 69−76. doi: 10.1016/j.tmrv.2017.10.001. Epub 2017 Oct 16. PMID: 29157918. Dostupné z https://pubmed.ncbi.nlm. nih.gov/29157918.
De Carolis MP. Use of protein C concentrate in neonatal period. Minerva Pediatr 2010; 62(3 Suppl 1): 29−30. PMID: 21089715. Dostupné z https://pubmed.ncbi.nlm.nih.gov/21089715.
De Kort EH, Vrancken SL, van Heijst AF, Binkhorst et al. Longterm subcutaneous protein C replacement in neonatal severe protein C deficiency. Pediatrics 2011; 127(5): e1338−e1342. doi: 10.1542/peds.2009-2913. Epub 2011 Apr 11. PMID: 21482600. Dostupné z https://pubmed.ncbi.nlm.nih.gov/21482600.

Findley T, Patel M, Chapman J, Brown D, Duncan AF. Acquired versus congenital neonatal purpura fulminans: A case report and literature review. J Pediatr Hematol Oncol 2018; 40(8): 625−627. doi: 10.1097/MPH.0000000000001150. Dostupné z https://pubmed.ncbi.nlm.nih.gov/29683961.

Girardis M, David S, Ferrer R. et al. Understanding, assessing and treating immune, endothelial and haemostasis dysfunctions in bacterial sepsis. Intensive Care Med 2024; 50: 1580–1592. https://doi.org/10.1007/s00134-024-07586-2. Dostupné z: https:// link.springer.com/article/10.1007/s00134-024-07586-2.
Chalmers E, Cooper P, Forman K, Mumford A. et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood 2011; 96 (11): 1066–1071. doi:10.1136/adc. 2010.199919. Epub 2011 Jan 12. PMID: 21233082. Dostupné z https://pubmed.ncbi.nlm.nih.gov/21233082.
Irfan Kazi SG, Siddiqui E, Habib I, Tabassum S, Afzal B, et al. Neonatal purpura fulminans, a rare genetic disorder due to protein C deficiency: A case report. J Pak Med Assoc 2018; 68(3): 463−465. PMID: 29540887. Dostupné z: https://pubmed.ncbi. nlm.nih.gov/29540887.
Perera TB, Murphy-Lavoie HM. Purpura fulminans. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2024. Dostupné z: https://www.ncbi.nlm.nih. gov/books/NBK532865/.
Siffel C, Wadhwa A, Tongbram V, Ogongo M, Sliwka H, et al. Comprehensive literature review of protein C concentrate use in patients with severe congenital protein C deficiency. Research and Practice in Thrombosis and Haemostasis 2024; 8(6): 102542. ISSN 2475-0379. Dostupné z: https://doi.org/10.1016/j. rpth.2024.102542.
Theron A, Dautremay O, Boissier E, Zerroukhi A, Baleine J, et al. Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review. Blood Adv 2022; 6(2): 495−502. doi: 10.1182/bloodadvances.2021005126. PMID: 34788405; PMCID: PMC8791598.

Labels

Neonatology Neonatal Nurse

EDITORIAL

Transfusion therapy in neonatology

Use of albumin in newborns

Hemostasis disorders in neonates – a practical approach

Vitamin K in neonatology

Anemia of prematurity (AOP)

The importance of iron for newborns

Spontaneous aortic thrombosis in the neonatal period: Case study

Article was published in

Czech and Slovak Neonatology

2024 Issue 2