Purpura fulminans from reduced protein S following cytomegalovirus and varicella infection

Fonkoua, Lionel Aurelien Kankeu; Zhang, Simin; Canty, Ethan; Fairfull, Aubree; Benich, Sarah; Knab, Andrea; Polimera, Hyma; Songdej, Natthapol

doi:10.1002/ajh.25386

Cited by 5 publications

(3 citation statements)

References 26 publications

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“…Viruses including Ebola, Marburg, Dengue, Huaiyangshan virus and Crimean-Congo hemorrhagic fever (CCHF) give rise to hemorrhagic fever in the host via multiple mechanisms, including cytokine-mediated endothelial activation and damage, clotting factor consumption and direct hepatic toxicity causing decreased production of clotting factors [42]. Hemorrhagic complications have rarely been noted in infections with non-hemorrhagic viruses such as varicella zoster virus (VZV) [42][43][44][45], cytomegalovirus (CMV) [46][47][48] and Epstein-Barr virus (EBV) [49]. On the other end of the spectrum, thrombotic complications including deep vein thrombosis (DVT), pulmonary embolism (PE), thrombotic microangiopathy (TMA), thrombotic thrombocytopenic purpura (TTP) and portal vein thrombosis have been reported in many viral infections including Avian influenza (H5N1), Swine flu (H1N1), Parvovirus B19, CMV, EBV, VZV, Hepatitis A and Hepatitis C virus and human immunodeficiency virus (HIV) [50].…”

Section: Viral Infection Inflammation and Thrombosismentioning

confidence: 99%

Hyperthrombotic Milieu in COVID-19 Patients

Kamel

Yin

Zavaro

et al. 2020

Cells

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COVID-19 infection has protean systemic manifestations. Experience from previous coronavirus outbreaks, including the current SARS-CoV-2, has shown an augmented risk of thrombosis of both macrovasculature and microvasculature. The former involves both arterial and venous beds manifesting as stroke, acute coronary syndrome and venous thromboembolic events. The microvascular thrombosis is an underappreciated complication of SARS-CoV-2 infection with profound implications on the development of multisystem organ failure. The telltale signs of perpetual on-going coagulation and fibrinolytic cascades underscore the presence of diffuse endothelial damage in the patients with COVID-19. These parameters serve as strong predictors of mortality. While summarizing the alterations of various components of thrombosis in patients with COVID-19, this review points to the emerging evidence that implicates the prominent role of the extrinsic coagulation cascade in COVID-19-related coagulopathy. These mechanisms are triggered by widespread endothelial cell damage (endotheliopathy), the dominant driver of macro- and micro-vascular thrombosis in these patients. We also summarize other mediators of thrombosis, clinically relevant nuances such as the occurrence of thromboembolic events despite thromboprophylaxis (breakthrough thrombosis), current understanding of systemic anticoagulation therapy and its risk–benefit ratio. We conclude by emphasizing a need to probe COVID-19-specific mechanisms of thrombosis to develop better risk markers and safer therapeutic targets.

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Section: Viral Infection Inflammation and Thrombosismentioning

confidence: 99%

Hyperthrombotic Milieu in COVID-19 Patients

Kamel

Yin

Zavaro

et al. 2020

Cells

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“…Post-infectious purpura fulminans is rare and develops during the recovery period from group A streptococcal infection, varicella-zoster, or rarely, from human herpesvirus 6 infection. 3,4 Postinfectious purpura fulminans begins 7 to 10 days after the onset of the initial infection and correlates with the production of infection-triggered antibodies to protein S. 4,5 As protein S is a co-factor of protein C during anti-coagulation this could explain the exuberance of the clinical findings in a patient with a partial protein C deficiency.…”

Section: Discussionmentioning

confidence: 99%

Purpura Fulminans in a 20-Year-Old Female

Alves

Brites

Coutinho

2021

SPDV

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A previously healthy 20-year-old female presented with extensive retiform purpura located at the face, upper and lower limbs, one week after an episode of acute tonsillitis. Despite the exuberance of the cutaneous findings and progression to skin necrosis she had no accompanying symptoms. Laboratory investigation revealed a heterozygous protein C mutation (exon 9, c.1332G> C, p.Trp444Cys), accounting for a partial deficiency of this anticoagulant protein. The patient was started on broad spectrum antibiotics, anticoagulation and systemic corticosteroids, with no lesional progression and complete resolution of cutaneous ulceration within 6 months. This is a singular case of purpura fulminans, since two different causative factors precipitated the events. The previous tonsillitis reported by the patient is significant, because the serum concentration of protein S may also decrease after an infectious event - post-infectious purpura fulminans. This case illustrates that purpura fulminans due to autoantibodies against protein S, although rare, should be considered, especially in the absence of a severe acute infection. It also illustrates how in a given patient different independent factors can act simultaneously, triggering potentially devastating clinical scenarios.

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“…[ 3 ] Hence, the borderline B cell deficiency could have triggered the endothelial cell damaging effect of CMV infection and played a synergistic role in PF. Fonkoua et al [ 4 ] reported a patient having PF due to protein S deficiency post cytomegalovirus and varicella infection. Boger[ 5 ] reported portal vein thrombosis in a 4-month-old girl due to the presence of CMV infection and inherited protein c and s deficiency.…”

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confidence: 99%