Autoimmune Protein S Deficiency in a Boy with Severe Thromboembolic Disease

D’Angelo, Armando; Valle, Patrizia Della; Crippa, L.; Pattarini, Elisabetta; Lm, Grimaldi; S, Viganò D'Angelo

doi:10.1056/nejm199306173282405

Cited by 97 publications

(58 citation statements)

References 32 publications

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“…These include nephrotic syndrome, disseminated intravascular coagulation, liver disease, and the use of drugs such as oral anticoagulants (warfarin) and L-asparaginase. 147,161 Autoimmune PS deficiency can also develop following an infection (such as chickenpox and HIV) [162][163][164] or in association with multiple myeloma. 165 Here, skin necrosis and purpura fulminans are severe manifestations and begin 7 to 10 days after the onset of the precipitating infection.…”

Section: Acquired Ps Deficiencymentioning

confidence: 99%

Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S–C4b binding protein complex

Rezende

Simmonds

Lane

2004

Blood

192

171

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Section: Acquired Ps Deficiencymentioning

confidence: 99%

Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S–C4b binding protein complex

Rezende

Simmonds

Lane

2004

Blood

192

171

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“…Subsequently, acquired free PS deficiency was described in children and adults with acute VZV infection and PFϮTE (2)(3)(4)(5)(6)(7)(8). We reported the LA and free PS deficiency in seven previously healthy children with acute VZV infection complicated by PFϮTE (6).…”

mentioning

confidence: 90%

The Varicella-Autoantibody Syndrome

et al. 2001

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This cross-sectional study was conducted to determine the incidence of autoantibodies to phospholipids and coagulation proteins in children with acute varicella zoster virus (VZV) infection. Study groups included children with VZV alone or complicated by purpura fulminans and/or thromboembolism. VZV naïve children and children who had VZV Ͼ1 y before sample collection formed a control group. Blood was assayed for the following: free protein S (PS), protein C, antithrombin, and prothrombin; antibody binding to these proteins; lupus anticoagulant; anticardiolipin antibody; antiphospholipid antibodies; and prothrombin fragment 1ϩ2. Data regarding coinfections was collected. Forty-three VZV-infected children showed an increased frequency of lupus anticoagulant, anticardiolipin antibody, antiphospholipid antibodies, and autoantibodies to PS, protein C, prothrombin, and antithrombin in comparison to 52 children without acute VZV (p Ͻ 0.0001). Seventeen children with VZV and purpura fulminans and/or thromboembolism showed a statistically significant decrease in free PS, significantly increased PS IgG antibody, and significantly increased prothrombin fragment 1ϩ2 (p Ͻ 0.0001) compared with the group without acute VZV and the group with uncomplicated VZV. Twenty-six children with uncomplicated VZV showed In a 1990 review, Francis (1) reported the association of VZV and/or streptococcal infection in 30% of children with idiopathic purpura fulminans. Subsequently, acquired free PS deficiency was described in children and adults with acute VZV infection and PFϮTE (2-8). We reported the LA and free PS deficiency in seven previously healthy children with acute VZV infection complicated by PFϮTE (6). We previously found evidence suggestive of an autoimmune cause of acquired PS deficiency.We now report a cross-sectional study designed to determine the prevalence of autoantibodies directed against a variety of phospholipids and coagulation proteins in previously healthy children with VZV infection. The aim of this study was to compare results obtained on blood samples from children with uncomplicated acute VZV infection with samples from children with acute VZV complicated by symptomatic PFϮTE. It was hypothesized that coagulation abnormalities in the children with symptomatic PFϮTE would be more prevalent and more severe but not intrinsically different from those found in healthy children with uncomplicated VZV infection. METHODS Clinical MethodsThis cross-sectional study was performed with the approval of the Colorado Multi-Institutional Review Board and the Pediatric Clinical Research Center at The Children's Hospital of Denver. Informed consent and assent forms were signed before enrollment in the study.

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“…More seldom, autoantibodies may develop against components of the hemostasis system. Some of them neutralize proteins involved in the regulation of thrombus formation, causing acquired thrombotic tendencies due to autoantibodies inactivating naturally occurring anticoagulants such as protein C and protein S, 1,2 or the von Willebrand factor cleaving protease ADAMTS13. 3,4 On the other hand, autoantibodies directed against procoagulant factors cause a bleeding tendency, such as acquired hemophilia A due to the development of anti-factor VIII (FVIII) autoantibodies (autoantibodies against procoagulant factors other than FVIII are rare).…”

mentioning

confidence: 99%