title: Refractory thrombotic thrombocytopenic purpura and membranoproliferative glomerulonephritis successfully treated with rituximab: a case associated with hepatitis C virus infection.
abstract: 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.
authors.affiliation: Renal Unit, Department of Medicine and Geriatrics, Kwong Wah Hospital, Kowloon, Hong Kong. maksk@ha.org.hk
authors.forename: S K
authors.initials: SK
authors.lastname: Mak
authors.forename: K Y
authors.initials: KY
authors.lastname: Lo
authors.forename: M W
authors.initials: MW
authors.lastname: Lo
authors.forename: S F
authors.initials: SF
authors.lastname: Chan
authors.forename: K C
authors.initials: KC
authors.lastname: Lo
authors.forename: Y Y
authors.initials: YY
authors.lastname: Wong
authors.forename: Gensy M W
authors.initials: GM
authors.lastname: Tong
authors.forename: P N
authors.initials: PN
authors.lastname: Wong
authors.forename: Edmond S K
authors.initials: ES
authors.lastname: Ma
authors.forename: Andrew K M
authors.initials: AK
authors.lastname: Wong
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001158:Arteries / Q000473:pathology; D015897:Comorbidity; D003404:Creatinine / Q000097:blood; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy* / Q000453:epidemiology* / Q000473:pathology; D016174:Hepacivirus / Q000235:genetics; D006526:Hepatitis C / Q000453:epidemiology*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000473:pathology; D008099:Liver / Q000473:pathology; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000453:epidemiology* / Q000628:therapy; D012367:RNA, Viral / Q000097:blood; D000069283:Rituximab; D017211:Treatment Failure
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D012367:RNA, Viral; D000069283:Rituximab; D003404:Creatinine; D000410:Alanine Transaminase; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human