Refractory thrombotic thrombocytopenic purpura and membranoproliferative glomerulonephritis successfully treated with rituximab: A case associated with hepatitis C virus infection

Renal Unit, Department of Medicine and Geriatrics, Kwong Wah Hospital, Kowloon, Hong Kong.
Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine (Impact Factor: 0.87). 07/2009; 15(3):201-8.
Source: PubMed


Plasmapheresis remains the main treatment modality for patients with thrombotic thrombocytopenic purpura. We report a patient who had simultaneous onset of membranoproliferative glomerulonephritis and thrombotic thrombocytopenic purpura. She did not improve after 48 plasmapheresis sessions. A 6-week course of weekly intravenous doses of rituximab was then given. This achieved complete remission of her nephrotic syndrome and improvement in her renal function, so plasmapheresis was ceased. She had a low ADAMTS13 antigen level and a positive ADAMTS13 antibody, both of which reverted to normal after treatment with rituximab. This coincided with a rise in her hepatitis C virus RNA and liver transaminases. Liver biopsies did not reveal active fibrosis. Her hepatitis C virus RNA titre dropped afterwards, and she had no relapses of her thrombotic thrombocytopenic purpura and nephrotic syndrome, for more than 2 years after remission. The simultaneous onset and successful outcomes of both the membranoproliferative glomerulonephritis and thrombotic thrombocytopenic purpura illustrate the usefulness of rituximab. We discuss its use and risks, in the context of chronic hepatitis C infection.

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    • "An earlier case of TTP with membranoproliferative glomerulonephritis with Hepatitis C infection was successfully treated with plasmapheresis and Rituximab. However in that case patient was not treated with any interferon therapy in the past and treatment with Rituximab had resulted in the elevation of liver transaminase.15 Acute refractory TTP in a patient of chronic hepatitis C, on interferon therapy, successfully treated with weekly Rituximab along with plasmapheresis and steroids during the first episode of TTP have not been reported previously in literature. "
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    ABSTRACT: Thrombotic thrombocytopenic purpura (TTP) is a life threatening condition associated with formation of platelet thrombi. Deficiency of ADAM TS 13 with presence of inhibitory anti-ADAM TS 13 Immunoglobulin G antibody is seen in patients with acquired TTP. TTP in patients on interferon therapy for chronic hepatitis C has rarely been reported. Furthermore, successful treatment of an initial episode of acute refractory acquired TTP, in a patient of chronic hepatitis C during interferon therapy with Rituximab, has not been previously reported. Here we describe a case of acute refractory acquired TTP associated with pegylated interferon therapy for her chronic hepatitis C infection. Initially refractory to plasmapheresis and steroids, she was successfully treated with Rituximab and plasmaphersis without any evidence of reactivation of hepatitis.
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    • "One patient with HCV-related liver cirrhosis was complicated by fetal TTP following the development of an ADAMTS13 inhibitor [15]. In addition, a simultaneous refractory TTP and membranoproliferative glomerulonephritis associated with HCV infection and successfully treated with rituximab have been also reported, without any apparent relation to IFN therapy [16] "
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