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She was initially treated with antibiotics under the impression of early-onset neonatal sepsis; however, her lesions worsened and rapidly progressed. The protein C chromogenic activity was remarkably decreased at below 10%, similar to a protein C antigen level below 1%, highly suggestive of severe congenital protein C deficiency. Molecular analysis revealed compound heterozygous likely pathogenic variants in the protein C gene. After the diagnosis, fresh frozen plasma (FFP) transfusions every 8\u2009hours until the patient was stabilized and therapeutic low-molecular-weight heparin was started. She developed complications related to FFP administration, such as acute kidney injury, hypertension and proteinuria, during the interim period, awaiting the initiation of protein C concentrate replacement therapy.<\/jats:p>","DOI":"10.1136\/bcr-2024-263471","type":"journal-article","created":{"date-parts":[[2025,5,2]],"date-time":"2025-05-02T07:46:23Z","timestamp":1746171983000},"page":"e263471","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Compound heterozygous congenital protein C deficiency: a challenging management with recurrent purpura fulminans treated with protein C concentrations"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1951-4365","authenticated-orcid":false,"given":"Chonlatis","family":"Srichumpuang","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Pediatric Hematology and Oncology, Department of Pediatrics, King Chulalongkorn Memorial Hospital, Bangkok, Thailand"},{"name":"Center of Exellence in Pediatric Hematology\/Oncology, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Phawin","family":"Kor-anantakul","sequence":"additional","affiliation":[{"name":"Center of Excellence for Medical Genomics, Medical Genomics Cluster, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand"},{"name":"Excellence Center for Genomics and Precision Medicine, King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonardo R","family":"Brandao","sequence":"additional","affiliation":[{"name":"Division of Haematology\/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darintr","family":"Sosothikul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology and Oncology, Department of Pediatrics, King Chulalongkorn Memorial Hospital, Bangkok, Thailand"},{"name":"Center of Exellence in Pediatric Hematology\/Oncology, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,5,2]]},"reference":[{"key":"2025082803184351000_18.5.e263471.1","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1055\/s-0038-1657550","article-title":"Protein C deficiency: from gene defects to disease","volume":"78","author":"Reitsma","year":"1997","journal-title":"Thromb Haemost"},{"key":"2025082803184351000_18.5.e263471.2","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1055\/s-0038-1653885","article-title":"Protein C deficiency: a database of mutations, 1995 update. 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