RESUMO
Purpura fulminans and venous thrombosis are rare complications of chickenpox. We report the case of a 6 year old with no history individuals who experienced cerebral thrombophlebitis, 3 weeks after varicella. MRI, performed at admission, has objectified longitudinal sinus thrombosis and a frontal parenchymal hematoma law. Meanwhile, a recent varicella seroconversion was demonstrated. The assessment of thrombophilia, meanwhile, has objectified a significant decrease in free protein S and activity, without associated DIC. Origin acquired this deficit was confirmed by the detection of antibodies (IgG and IgM) against the total protein S by ELISA. After evaluation of the benefit/risk only anticoagulation was initiated. The clinical and biological evolution was favorable, with rapid normalization of the S protein and decrease of anti-protein S. Many studies report the presence of anti-protein S in young children at the waning of chickenpox, without their exact frequency is determined. The decrease in protein S they cause leads to a transient hypercoagulable state may result in different clinical pictures. Cases of purpura fulminans seem more frequent when venous thrombosis isolated post chickenpox, sometimes atypical, appear rare.
Assuntos
Varicela/complicações , Proteína S/imunologia , Tromboflebite/complicações , Anticorpos/sangue , Varicela/sangue , Varicela/imunologia , Criança , Feminino , Humanos , Trombose Intracraniana/sangue , Trombose Intracraniana/complicações , Deficiência de Proteína S/sangue , Deficiência de Proteína S/complicações , Deficiência de Proteína S/imunologia , Tromboflebite/sangue , Vasculite do Sistema Nervoso Central/sangue , Vasculite do Sistema Nervoso Central/complicaçõesRESUMO
Purpura fulminans usually consists of large, often symmetrical, spreading ecchymosis, which may later develop into extensive areas of skin necrosis and peripheral gangrene. Postinfectious purpura fulminans associated with an autoantibody directed against protein S has been described. The interaction and the contribution of recently described mutations such as factor V Leiden and prothrombin G20210A to the development and progression of postinfectious purpura fulminans and venous thrombosis is not known. The authors describe a patient heterozygous for prothrombin G20210A who developed purpura fulminans and extensive venous thrombosis secondary to acquired protein S deficiency.
Assuntos
Vasculite por IgA/etiologia , Deficiência de Proteína S/complicações , Deficiência de Proteína S/imunologia , Protrombina/efeitos adversos , Protrombina/genética , Autoanticorpos/efeitos adversos , Autoanticorpos/sangue , Pré-Escolar , Heterozigoto , Humanos , Vasculite por IgA/genética , Vasculite por IgA/imunologia , Masculino , Mutação de Sentido Incorreto , Deficiência de Proteína S/diagnóstico , Trombose Venosa/etiologia , Trombose Venosa/terapiaRESUMO
Pneumonia is the most common serious complication of varicella infection in adults. A variety of thrombotic complications including purpura fulminans and disseminated intravascular coagulation have been reported in children with varicella but not in adults. Two men with varicella pneumonia who had profound lower extremity ischemia caused by thrombosis of the profunda femoris and tibial arteries are reported. Both patients had free protein S deficiency and vascular thrombosis in association with varicella pneumonia without overt evidence of disseminated intravascular coagulation or purpura fulminans. Antiphospholipid immunoglobulin G and immunoglobulin M antibodies were present in one, whereas the other had evidence of the lupus anticoagulant. The proposed pathogenesis and management options including intraarterial thrombolytic therapy with urokinase and the need for long-term anticoagulation are discussed.
Assuntos
Varicela/complicações , Artéria Femoral , Pneumonia Viral/complicações , Deficiência de Proteína S/complicações , Trombose/etiologia , Artérias da Tíbia , Adulto , Anticorpos Antifosfolipídeos/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Ativadores de Plasminogênio/uso terapêutico , Deficiência de Proteína S/sangue , Deficiência de Proteína S/imunologia , Radiografia , Trombose/diagnóstico por imagem , Trombose/tratamento farmacológico , Ativador de Plasminogênio Tipo Uroquinase/uso terapêuticoRESUMO
Investigation of recurrent venous thromboembolic events in a 46-year-old man with progressive IgG kappa (total serum IgG, 74.3 mg/ml) multiple myeloma revealed profound reductions in free protein S (PS) antigen (<0.l U/ml) and PS activity (0.33 U/ml). Total PS antigen, protein C, antithrombin III, and C4b-binding protein levels were within normal limits. The patient had no family history suggestive of a congenital PS deficiency and no history of thrombosis predating the diagnosis of his plasma cell dyscrasia. Patient IgG was isolated from serum using a protein A-sepharose affinity column and characterized. PS-dependent clotting assays (Staclot Protein S, Diagnostica Stago, Asnieres sur-Seine, France) performed on normal pooled plasma mixed with dilutions of patient IgG (0.0-33.0 mg/ml) revealed a dose-dependent neutralization of PS activity by 43%. Total and free PS antigen levels were measured using Laurell rocket electroimmunodiffusion (Assera-Plate Protein S, Diagnostica Stago), which revealed a similar dose-dependent reduction in free PS antigen but preserved normal total PS antigen. Free PS antigen was reduced by 77% to 0.23 U/ml using an IgG concentration (16.5 mg/ml) less than one-fourth of that of the patient at time of serum collection. Specific binding of the patient IgG to commercially available purified human PS was demonstrated by Western immunoblot analysis. Whereas acquired free PS deficiency has been previously reported in association with nephrotic syndrome, inflammatory bowel disease, HIV infection, and varicella infection, this is the first reported case of a hypercoagulable syndrome associated with acquired free PS deficiency and multiple myeloma.