Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection

Abstract

Acquired protein S deficiency and lupus anticoagulant have been described in children with varicella who had purpura fulminans, disseminated intravascular coagulation or thrombosis. The aim of this study was to investigate the natural anticoagulants, hypercoagulability markers, other parameters of coagulation and fibrinolytic systems, and the presence of the lupus anticoagulant in otherwise healthy children with acute varicella infection. Blood samples were obtained from 17 children with varicella without thrombosis during acute varicella infection and 1 mo after onset. Coagulation tests included determinations of the prothrombin time, the activated partial thromboplastin time, the thrombin time, the thrombin antithrombin complex, the prothrombin fragment F 1 + 2, the tissue plasminogen activator, the plasminogen activator inhibitor‐1, protein C activity and free protein S antigen. Antiphospholipid antibodies were determined in enzyme‐linked immunosorbent assays. The mean free protein S concentration in the acute phase (0.63 ±0.16U/ml) was significantly lower than that of the concentration determined 1 mo later (0.82 ± 0.17 U/ml). The children with acquired free protein S deficiency also had a lupus anticoagulant. Elevated concentrations of the prothrombin fragment F 1+2, the thrombin antithrombin complex, D‐Dimer, tissue plasminogen activator and plasminogen activator inhibitor‐1 were detected in most of the children. Conclusion: There is a significantly increased prevalence of lupus anticoagulant, a significantly reduced plasma concentration of free protein S and elevations in coagulation and fibrinolytic parameters in otherwise healthy children with acute varicella infection.

Clinical significance of different antiphospholipid antibodies in the WAPS (warfarin in the antiphospholipid syndrome) study

“…27 They are commonly transient and present together with LAs, and only in a few cases they cause purpura fulminans. 28 These clinical scenarios are very selective and unusual, which makes the routine detection of IgM aPS antibodies of little use in adult patients suspected of suffering from APS. Even more, the presence of aPS antibodies of both isotypes seems to have a protective effect against anamnestic thrombosis.…”

Varicella-Associated Purpura Fulminans: Chicken Pox Is Not Always Benign

“…Patients with severe PF or disseminated intravascular coagulation associated with varicella usually have very low or undetectable PS concentrations. While children with uncomplicated varicella have mild free-PS defi ciency [8] , our patient had a very low PS level of 13%, which could explain the severity of the clinical manifestation. PF is often fatal and survivors may have considerable morbidity related to loss of digits, limbs, or areas of the skin -all of which was the case in our patient.…”

“…Transient defi ciencies of PS and PC, which returned to normal after 3 months, were present in our patient. Acquired PS and PC defi ciency could be a consequence of disseminated intravascular coagulation or result from the presence of a specifi c antibody against the protein [4,8,9] . Patients with severe PF or disseminated intravascular coagulation associated with varicella usually have very low or undetectable PS concentrations.…”

Coagulopathy secondary to chicken pox: a case report

“…Protein S deficiency secondary to varicella complicated by thrombotic and vascular events namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke has been reported. 8,9 Besides the hemorrhagic skin lesions, hemorrhagic complications like bleeding gums, hemoptysis, pulmonary and gastrointestinal bleeding, intra-cerebral haemorrhage, disseminated intravascular coagulation have been infrequently reported in children when it is called hemorrhagic chicken pox. 3 There have been very few case reports in healthy immunocompetent adults.…”