title: Two cases of kidney transplantation-associated thrombotic microangiopathy successfully treated with eculizumab.
abstract: Transplantation-associated thrombotic microangiopathy (TA-TMA) is relatively rare and requires immediate intervention to avoid irreversible organ damage or death; however, consensus regarding the treatment approach is lacking. Atypical haemolytic uraemic syndrome (aHUS) is a rare disease caused by dysregulation of the alternative complement pathway resulting in TMA. aHUS is histologically similar to TA-TMA; approximately 60% of TA-TMA patients have complement dysregulation. Eculizumab, a humanized anti-C5 monoclonal antibody, inhibits terminal membrane-attack complex formation and TMA progression. Eculizumab has been successfully used to treat aHUS post-transplant. We present two cases of kidney TA-TMA due to unknown causes, suspected antibody-mediated rejection, or calcineurin inhibitor (CNI)-related toxicity that developed on day 1 or 2 post-kidney transplantation. Low platelet count and haemoglobin level with red cell fragments were detected. Despite steroid pulse, plasma exchange (PE), and intravenous immunoglobulin therapy, TA-TMA did not improve; therefore, eculizumab was administered despite no genetic testing. Laboratory data, including renal function, improved immediately. TA-TMA treatment primarily involves PE initiation or CNI discontinuation; eculizumab can be used to safely treat TA-TMA and then be ceased in the short term. Therefore, eculizumab administration might be beneficial for kidney TA-TMA as early as the diagnosis of refractory to PE.
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ikeda
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Okumi
authors.affiliation: Internal Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kohei
authors.initials: K
authors.lastname: Unagami
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Taichi
authors.initials: T
authors.lastname: Kanzawa
authors.affiliation: Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Anri
authors.initials: A
authors.lastname: Sawada
authors.affiliation: Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kunio
authors.initials: K
authors.lastname: Kawanishi
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kazuya
authors.initials: K
authors.lastname: Omoto
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Ishida
authors.affiliation: Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kazunari
authors.initials: K
authors.lastname: Tanabe
mesh_terms: D000328:Adult; D000368:Aged; D064591:Allografts; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001706:Biopsy; D003167:Complement Activation / Q000187:drug effects; D051056:Complement Inactivating Agents / Q000494:pharmacology*; D005260:Female; D006801:Humans; D007668:Kidney / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D012074:Remission Induction; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; C481642:eculizumab
keywords: atypical haemolytic uraemic syndrome; eculizumab; kidney transplantation; plasma exchange; thrombotic microangiopathies