Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis

Manco‐Johnson, Marilyn J.; Nuss, Rachelle; Key, Nigel S.; Moertel, Christopher L.; Jacobson, Linda; Meech, Sandra J.; Weinberg, Adriana; Lefkowitz, Jerry B.

doi:10.1016/s0022-3476(96)70274-3

Cited by 176 publications

(133 citation statements)

References 12 publications

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“…This study indicated that not only meningococcal disease but also other severe infections might play a role in PF (Table I). Although we could not analyze autoantibodies against PC and PS, in our patient with varicella, an acquired PS deficiency was probably responsible for PF that was shown to be a mechanism in PF occurring after varicella [9,10]. However, in this patient, the concomitant presence of antiphospholipid antibodies might also have played a role in the development of PF.…”

Section: Discussionmentioning

confidence: 64%

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Outcome in children with purpura fulminans: Report on 16 patients

et al. 2005

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Purpura fulminans (PF) is a severe disorder of acute onset with high morbidity and mortality. In children, this rapidly progressive illness is usually associated with severe bacterial or viral infections. However, some other conditions may participate in the development of PF. Our objective was to investigate the underlying and associated disorders and the outcomes of the disease in 16 children, 7 males and 9 females ranging in age from 3.5 months to 12 years (median age, 2 years). Thirteen of the 16 children (81%) were 4 years of age or younger. The remaining 3 patients were 9, 10, and 12 years of age. Among these 13 infants and small children, 7 (43%) had infection, 2 infants had congenital cardiac disorders necessitating minor or major surgical intervention, and 1 infant and 3 children had different miscellaneous disorders. The factor V G1691A mutation was present in six of the 13 small children (46%). None of the 3 older children carried the mutation. Six (37.5%) of the 16 patients had protein C deficiencies, and 9 (56%) had protein S deficiencies. These deficiencies, except one for protein S, were acquired. Ten patients except two who were diagnosed at this center were treated with fresh frozen plasma. They were also given heparin. Nine (69%) of the 13 children 4 years of age or younger and one of the older children (33%) required amputation. Five of the six patients (83%) who had factor V G1691A mutation, and who also exhibited severe infection, required amputation. This study suggests that an age of 4 years or less is a risk factor for the development of PF during severe infections, especially in the presence of factor V G1691A mutation and congenital heart disease, necessitating major or minor surgical interventions. This study also shows that the amputation rate in 10 patients, after excluding the patients who had been referred to our center after development of sequelae, was 60%. The survival rate among these 10 patients may indicate that, with the treatment protocol, PF need not be regarded as a lethal disease any more. It is also suggested that effective immunization programs and better health care have probably resulted in some changes in the etiological profile of PF. Am. J. Hematol. 80:20–25, 2005. © 2005 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 64%

“…The antibodies interfere with the function of these inhibitors of the coagulation system. In the post-infectious cases that have been observed mainly in older children and adults, development of PF appears to be due to the inhibition of PC and/or PS by auto-antibodies [9,10].…”

Section: Introductionmentioning

confidence: 99%

Outcome in children with purpura fulminans: Report on 16 patients

et al. 2005

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“…It has been demonstrated that aPL can be induced in patients infected with many bacteria, other microorganisms (35)(36)(37)(38)(39)(40)(41)(42)(43)(44), and viruses, including CMV (45), varicella zoster virus (46), human immunodeficiency virus (47), hepatitis C (48), and Epstein-Barr virus (49). In addition, transient LAC activity in patients with Epstein-Barr virus infections has been reported (49).…”

Section: Discussionmentioning

confidence: 99%

Antiphospholipid antibodies induced in mice by immunization with a cytomegalovirus‐derived peptide cause thrombosis and activation of endothelial cells in vivo

Gharavi

Pierangeli

Espinola

et al. 2002

Arthritis & Rheumatism

165

115

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Objective. To characterize the binding and functional properties of antiphospholipid antibodies (aPL) induced by immunization with a viral peptide and to determine whether aPL are pathogenic in vivo.Methods. Ten murine monoclonal aPL were generated from spleen cells of PL/J mice immunized with TIFI, a phospholipid-binding peptide spanning Thr 101 -Thr 120 of ULB0-HCMVA from human cytomegalovirus (CMV), which shares structural similarity with the phospholipid-binding site of ␤ 2 -glycoprotein I (␤ 2 GPI).Results. The antibodies generated had aPL activity that was inhibited by cardiolipin liposomes, and this inhibition was enhanced in the presence of ␤ 2 GPI. Some of the antibodies exhibited binding to cultured endothelial cells in vitro, and some had lupus anticoagulant activity. Injection with 2 of the monoclonal aPL in mice resulted in a significant increase in the number of leukocytes adhering to endothelial cells and enhanced thrombus formation in vivo.Conclusion. These results indicate that aPL induced by immunization with a phospholipid-binding CMV peptide are pathogenic in vivo. The results also suggest a mechanism (molecular mimicry) by which pathogenic aPL may be generated in patients with antiphospholipid syndrome.

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“…Although varicella may increase the risk of stroke due to nonspecific inflammation and transient thrombophilia, most notably protein S deficiency (29,30), the predominant pathophysiology of varicella-associated stroke is likely VZV vasculopathy, similar to that in adults.…”

Section: Vzv Vasculopathy In Childrenmentioning

confidence: 99%

Varicella Zoster Virus: A Common Cause of Stroke in Children and Adults

Amlie‐Lefond

Gilden

2016

Journal of Stroke and Cerebrovascular Diseases

104

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Background-Varicella zoster virus (VZV) is a neurotropic exclusively human herpesvirus. Primary infection causes varicella (chickenpox), after which virus become latent in ganglionic neurons along the entire neuraxis. As cell-mediated immunity to VZV declines with advancing age and immunosuppression, VZV reactivates to produce zoster (shingles). One of the most serious complications of zoster is VZV vasculopathy.

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Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis

Cited by 176 publications

References 12 publications

Outcome in children with purpura fulminans: Report on 16 patients

Outcome in children with purpura fulminans: Report on 16 patients

Antiphospholipid antibodies induced in mice by immunization with a cytomegalovirus‐derived peptide cause thrombosis and activation of endothelial cells in vivo

Varicella Zoster Virus: A Common Cause of Stroke in Children and Adults

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