ABSTRACT
A child with vertical transmission of human immunodeficiency virus refractory to therapy developed zoster-induced protein S deficiency and recurrent strokes. Extensive carotid arteritis was found postmortem. The carotid tissue was positive for herpes varicella zoster by polymerase chain reaction, as were immunofixation stains of the arterial wall.
Subject(s)
Acquired Immunodeficiency Syndrome/diagnosis , HIV-1/pathogenicity , Herpesvirus 3, Human/pathogenicity , Infectious Disease Transmission, Vertical , Varicella Zoster Virus Infection/diagnosis , Vasculitis, Central Nervous System/diagnosis , Acquired Immunodeficiency Syndrome/drug therapy , Acquired Immunodeficiency Syndrome/transmission , Acquired Immunodeficiency Syndrome/virology , Adult , Anti-HIV Agents/therapeutic use , Antiretroviral Therapy, Highly Active , Carotid Arteries/pathology , Carotid Arteries/virology , Child, Preschool , Drug Tolerance , Fatal Outcome , Female , HIV-1/growth & development , Herpesvirus 3, Human/growth & development , Humans , Protein S Deficiency/diagnosis , Protein S Deficiency/pathology , Protein S Deficiency/virology , Recurrence , Stroke/diagnosis , Stroke/pathology , Stroke/virology , Varicella Zoster Virus Infection/pathology , Varicella Zoster Virus Infection/virology , Vasculitis, Central Nervous System/pathology , Vasculitis, Central Nervous System/virologyABSTRACT
Risk of cerebrovascular disease is increased in patients with HIV infection; however, cerebral venous sinus thrombosis is not commonly reported in this condition. We report a case of young man with intracranial hemorrhage whose radiologic investigations revealed thrombosis of the left transverse and sigmoid sinuses extending into the left internal jugular vein. On further investigations, he was found to be positive for HIV-1, and his procoagulant workup showed protein S deficiency. He was started on anticoagulant therapy, after which he showed gradual recovery. This case highlights a rarely encountered presentation of HIV infection as cerebral venous sinus thrombosis.
Subject(s)
HIV Infections/diagnosis , Sinus Thrombosis, Intracranial/etiology , Adult , HIV Infections/complications , Humans , Male , Protein S Deficiency/complications , Protein S Deficiency/diagnosis , Protein S Deficiency/virology , Sinus Thrombosis, Intracranial/diagnosisABSTRACT
BACKGROUND: Varicella zoster virus (VZV) infection can cause temporary acquired protein S or C deficiency via cross reacting antibodies and consequently inducing a hypercoagulable state. CASE DESCRIPTION: A 6-year-old girl with a history of congenital cardiac disease was seen at an Emergency Department with acute chest pain, dyspnoea and fever, seven days after developing chicken pox. Diagnostic tests revealed massive infarction of the spleen, and a protein S and C deficiency. In addition, blood cultures revealed a Lancefield group A ß-haemolytic streptococcus (GABHS). The patient recovered fully after treatment with low molecular weight heparin and antibiotics. CONCLUSION: In this patient, septic emboli caused splenic infarction. Thromboembolic complications should be suspected in children with VZV who present with acute symptoms, in particular if bacterial superinfection is found.
Subject(s)
Chickenpox/complications , Embolism/complications , Herpesvirus 3, Human/pathogenicity , Splenic Infarction/etiology , Streptococcal Infections/complications , Acute Disease , Chickenpox/immunology , Child , Cross Reactions , Embolism/immunology , Female , Humans , Protein C Deficiency/etiology , Protein C Deficiency/immunology , Protein C Deficiency/virology , Protein S Deficiency/etiology , Protein S Deficiency/immunology , Protein S Deficiency/virology , Splenic Infarction/immunology , Splenic Infarction/virology , Streptococcal Infections/immunologySubject(s)
Brain Ischemia/etiology , Cerebral Arterial Diseases/virology , Herpes Zoster/complications , Herpesvirus 3, Human/pathogenicity , Peripheral Vascular Diseases/etiology , Thrombosis/etiology , Adult , Antiphospholipid Syndrome/virology , Brain Ischemia/virology , Cerebral Arterial Diseases/complications , Cerebral Arteries/virology , Hemianopsia/etiology , Hemiplegia/etiology , Humans , Male , Perceptual Disorders/etiology , Peripheral Vascular Diseases/virology , Protein S Deficiency/virology , Retinal Artery Occlusion/etiology , Retinal Artery Occlusion/virology , Thrombophilia/virology , Thrombosis/virologyABSTRACT
Postinfectious purpura fulminans is a rare disease. Varicella is one of the precipitating conditions and we recently observed such a case. The 4-year-old child was found to have a severe transient protein S deficiency. By enzyme-linked immunosorbent assay and surface plasmon resonance we first demonstrated that anti-protein S antibodies were present and also transient. Next we characterized the epitopes against which these antibodies were directed and found that they predominantly recognized the N-terminal part of protein S. Finally we showed by thrombography a transient dramatic hypercoagulable state as a result of thrombin being unregulated by the dynamic protein C inhibitory system: in vitro thrombin generation, in response to a low concentration of tissue factor, was almost insensitive to activated protein C up to 25 nmol L(-1) on day 4 while it was normally sensitive on day 42. For the first time, we demonstrated a temporal relationship between protein S deficiency, antibodies to protein S and hypercoagulability, thus supporting the pathogenic role of these antibodies.
