title: Thrombotic Microangiopathy as a Cause of Chronic Kidney Transplant Dysfunction: Case Report Demonstrating Successful Treatment with Eculizumab.
abstract: Atypical hemolytic uremic syndrome is a rare disease associated with genetic or acquired defects in complement regulation which frequently leads to renal failure. Disease often recurs early after kidney transplantation, leading to a rapid irreversible loss of function. Extrarenal features, such as hemolysis and thrombocytopenia, may not always occur, and diagnosis is made by demonstrating the classic features of thrombotic microangiopathy on renal biopsy. Eculizumab, a terminal complement inhibitor, has been used successfully to treat fulminant early recurrent disease after transplantation. We describe a case of disease recurrence presenting in the second year after transplantation with a gradual decline in function and the first report of eculizumab treatment for chronic thrombotic microangiopathy in a transplanted kidney. The resultant diagnostic challenges and successful response to eculizumab in this setting are discussed.
authors.affiliation: Renal Unit, Freeman Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, United Kingdom. Electronic address: zediqbal@hotmail.com.
authors.forename: Z
authors.initials: Z
authors.lastname: Iqbal
authors.affiliation: Department of Pathology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.
authors.forename: K
authors.initials: K
authors.lastname: Wood
authors.affiliation: NHS Blood and Transplant, Newcastle upon Tyne, United Kingdom; Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: V
authors.initials: V
authors.lastname: Carter
authors.affiliation: Renal Unit, Freeman Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: T H
authors.initials: TH
authors.lastname: Goodship
authors.affiliation: Renal Unit, Freeman Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.
authors.forename: A L
authors.initials: AL
authors.lastname: Brown
authors.affiliation: Renal Unit, Freeman Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: N S
authors.initials: NS
authors.lastname: Sheerin
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D016030:Kidney Transplantation; D008875:Middle Aged; D055031:Primary Graft Dysfunction / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012008:Recurrence; D057049:Thrombotic Microangiopathies / Q000188:drug therapy* / Q000209:etiology
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab