Severe Hemorrhage Secondary To IgA Vasculitis With Varicella Infection: A Case Report

Abstract

IgA vasculitis (IgAV), formerly named as Henoch Schönlein Purpura, is an IgA-mediated systemic small vessel vasculitis with skin, gastrointestinal tract, joints, and kidneys involvement. It is the most common vasculitis in childhood. Varicella-zoster virus-associated IgA vasculitis has been reported previously in few cases. However, IgA vasculitis complicated by chickenpox rarely occurred severe hemorrhagic manifestation and had not yet been reported. Herein, we present a case of an eight-year-old girl who developed IgA vasculitis complicated by disseminated intravascular coagulation and multiple organ dysfunction syndrome, including severe acute pancreatitis and acute liver failure secondary to varicella-zoster virus infection and finally underwent blood purification including hemoperfusion, plasmapheresis, and continuous renal replacement therapy. Tumors and other diseases affecting coagulation and platelet function, such as systemic lupus erythematosus, ANCA-associated vasculitis, primary thrombocytopenia, and secondary thrombotic microangiopathies, have been excluded during her hospitalization. Varicella was considered to be the cause of the severe complications. A possible mechanism for the occurrence of severe complications is that chickenpox infection induces the production of heparin-like substances disturbing normal coagulation function, and finally occurs uncontrolled hemorrhage manifestation including gastrointestinal hemorrhage, uncontrolled bleeding at the catheter placement and injection site. She eventually was discharged after undergoing 37 days of treatment by antibiotics, antiviral drugs, protamine neutralizing heparin therapy and various blood products transfusion including platelets, red cells, fresh frozen plasma, cryoprecipitate, fibrinogen, and prothrombin complex transfusions. At the end of a month follow-up, the patient showed the purpuric rashes had nearly faded and had no sign of complication including the gastrointestinal tract, liver, and pancreas. Renal function tests and routine analysis of blood were normal. Hematuria(3+~4+) and proteinuria(1+~2+) can be seen in her urinalyses. At the 5th month follow-up, her renal function test and her urinalysis were normal, and her oral steroids were stopped completely. On this occasion, we should reduce the dose of heparin or no heparin in blood purification for IgA vasculitis complicated by chickenpox to reduce the incidence of severe bleeding or death.