Purpura fulminans in congenital protein C deficiency successfully treated with fresh frozen plasma and thrombomodulin

Hayami, Takuma; Yamaguchi, Akihiko; Kato, Takeshi; Tanaka, Toshihiro; Nishizawa, Yuka; Yanagi, Takahide; Taga, Takashi; Matsumoto, Shimpei; Uchiumi, Takeshi; Fujimoto, Noriki

doi:10.1111/1346-8138.14194

Cited by 2 publications

(2 citation statements)

References 5 publications

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“…New anticoagulation medications provide possibilities for the management of this medical condition. Hayami T et al reported the first case of purpura fulminans in a case with congenital PC deficiency that was successfully treated with FFP and thrombomodulin [37]. Cisneros GS et al published a case report of a 17-year-old male patient with severe PC deficiency that was successfully managed with the use of a direct oral Xa inhibitor, rivaroxaban [32].…”

Section: Discussionmentioning

confidence: 99%

Clinical and genetic features of Chinese pediatric patients with severe congenital protein C deficiency who first presented with purpura fulminans: A case series study and literature review

Tang

Zhang

Yang

et al. 2022

Thrombosis Research

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Introduction: Purpura fulminans (PF) is a hematological emergency that can be caused by severe congenital protein C (PC) deficiency. It has been rarely reported in the Chinese population. We aimed to characterize the clinical and genetic features of Chinese pediatric patients with severe congenital PC deficiency who first presented with PF. Materials and methods: Twelve pediatric patients were diagnosed with severe congenital PC deficiency with PF, which was diagnosed based on our hospital records and previous reports from 1988 to July 2021 in China. We evaluated the clinical and genetic features of these patients. Results: Nine patients (9/12, 75%) had onsets that were observed within the first 48 h after birth. Six patients had a family history of thromboembolism. There was no consanguinity. Other symptoms were intracranial thrombosis or hemorrhage (4, 33.3%), ocular lesions (2, 16.7%), gastrointestinal hemorrhage (2, 16.7%) and kidney infarction before birth (1, 8.3%). All but one of the patients (one case not detected) had a plasma PC activity of <10%. The genetic study indicated that in the eight patients with inherited PC deficiency, two were homozygous, five were compound heterozygous and one was heterozygous for PC deficiency. Conclusion: This is the first and largest case series of Chinese pediatric patients with severe congenital PC deficiency who first presented with PF. It has been shown that treatment with both fresh frozen plasma and anticoagulants is recommended when PC concentrate is not easily available, especially in developing countries.

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Section: Discussionmentioning

confidence: 99%

Clinical and genetic features of Chinese pediatric patients with severe congenital protein C deficiency who first presented with purpura fulminans: A case series study and literature review

Tang

Zhang

Yang

et al. 2022

Thrombosis Research

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“…Treatment targeting the coagulation cascade abnormalities found in purpura fulminans is also considered standard, including platelet transfusion for severe thrombocytopenia (platelet count <50×10 9 /dl) and replacement of coagulation factors [1]. The combination of thrombomodulin (a cofactor for thrombin) and fresh frozen plasma has also been found to be effective in contributing to the resolution of purpura fulminans [6]. Isolated protein C supplementation is a newer method used to successfully restore anticoagulant factors consumed in the disease process [7].…”

Section: Discussionmentioning

confidence: 99%

Purpura Fulminans in an adult with Pseudomonas aeruginosa septicemia

Wells¹,

Kennon²

2019

Our Dermatol Online

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Purpura fulminans is a serious dermatological condition that can be a sequela of sepsis complicated by disseminated intravascular coagulopathy (DIC). This occurs most frequently in children with Neisseria meningitis or Streptococcus pneumoniae infection. Once diagnosed, treatment of the underlying infection, surgical debridement, and replacement of anticoagulation factors are recommended. Most patients suffer significant long-term morbidity, including amputations and sometimes death. We describe a rare case of an adult patient with Pseudomonal bacteremia leading to purpura fulminans. The patient's underlying infection was promptly treated and her lesions were debrided appropriately. However, correction of coagulopathy was not pursued. Despite this, amputations were avoided and the patient survived. This case demonstrates the need to be vigilant and open minded to the diagnosis of dermatological diseases with atypical presentations. Further, it highlights the chief importance of treating the underlying infection and providing proper wound care in septic purpura fulminans.

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Purpura fulminans in congenital protein C deficiency successfully treated with fresh frozen plasma and thrombomodulin

Cited by 2 publications

References 5 publications

Clinical and genetic features of Chinese pediatric patients with severe congenital protein C deficiency who first presented with purpura fulminans: A case series study and literature review

Clinical and genetic features of Chinese pediatric patients with severe congenital protein C deficiency who first presented with purpura fulminans: A case series study and literature review

Purpura Fulminans in an adult with Pseudomonas aeruginosa septicemia

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