Haploidentical Hematopoietic Stem Cell Transplant complicated by Atypical Hemolytic Uremic Syndrome and Kidney Transplant from the same donor with no Immunosuppression but C5 Inhibition

Background Atypical hemolytic uremic syndrome (aHUS) is life-threatening condition particularly when complicating allograft hematopoietic stem cell transplant (HSCT). In the past, the outcome was very poor with the majority of patients reaching end stage renal disease (ESRD) or dying with little or no chances of kidney transplant (KTx) due to the high risk of relapse. The availability of C5 inhibition has opened up significant therapeutic opportunities and has improved the outcome particularly if complement dysregulation (CD) is the underlying pathogenetic mechanism. Methods: We describe a peculiar case of a girl with aHUS complicating HSCT and her subsequent successful KTx received from the same donor thus performed without immunosuppression but anti C5 inhibition. Results Soon after HSCT performed for acute lymphoblastic leukemia, the patient developed a thrombotic microangiopathy (TA-TMA) due to CD and reached ESRD. After 2 years on dialysis the patient received a KTx from her father who was already the HSCT donor. Given the full chimerism, no immunosuppressive agent was prescribed except a short (2 days) course of steroids and eculizumab to prevent aHUS relapse. Nine months after the KTx the patient is well with normal renal function, no immunosuppression and continues eculizumab prevention of aHUS (1 infusion every 21 days). Conclusions All patients with TA-TMA should be screened for the causes of CD. C5 inhibition with eculizumab is an important therapeutic resource to manage this complication. When KTx is necessary, immunosuppression can be safely withhold in case of same donor for both grafts and documented full chimerism. Correspondence information: Ardissino Gianluigi, Center for HUS Control, Prevention and Management, Fondazione, IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda, 9, 20122 Milan, Italy, e-mail: ardissino@centroseu.org Authorship page Authorship: Ardissino Gianluigi: participated in the writing of the paper Cresseri Donata: participated in the writing of the paper Giglio Fabio: participated in the writing of the paper Onida Francesco.: participated in the writing of the paper Iannuzzella Francesco: participated in the writing of the paper Tel Francesca: participated in the writing of the paper Giussani Antenore: participated in the writing of the paper Messa Piergiorgio: participated in the writing of the paper Longhi Selena: participated in the writing of the paper Vincenti Daniele: participated in the writing of the paper Tedeschi Silvana: participated in the writing of the paper Cugno Massimo: participated in the writing of the paper Ciceri Fabio: participated in the writing of the paper Disclosure: The authors declare no conflicts of interest Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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