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Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplantation. Sep 10, 2018; 8(5): 122-141
Published online Sep 10, 2018. doi: 10.5500/wjt.v8.i5.122
Thrombotic microangiopathy after renal transplantation: Current insights in de novo and recurrent disease
Fedaey Abbas, Mohsen El Kossi, Jon Jin Kim, Ajay Sharma, Ahmed Halawa
Fedaey Abbas, Nephrology Department, Jaber El Ahmed Military Hospital, Safat 13005, Kuwait
Fedaey Abbas, Mohsen El Kossi, Jon Jin Kim, Ajay Sharma, Ahmed Halawa, Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom
Mohsen El Kossi, Doncaster Renal Unit, Doncaster Royal Infirmary, Doncaster DN2 5LT, United Kingdom
Jon Jin Kim, Nottingham Children Hospital, Nottingham NG7 2UH, United Kingdom
Ajay Sharma, Transplant Surgery, Royal Liverpool University Hospitals, Liverpool UK L7 8XP, United Kingdom
Ahmed Halawa, Department of Transplantation Surgery, Sheffield Teaching Hospitals, Sheffield S57AU, United Kingdom
Author contributions: Abbas F designed the study, performed data collection, and wrote the manuscript; El Kossi M, Kim JJ and Sharma A reviewed and edited the manuscript; Halawa A contributed to conceptualization, study design, supervision of data collection and reviewing and editing of the manuscript.
Conflict-of-interest statement: No conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Ahmed Halawa, FRCS (Gen Surg), FRSC, MD, MSc, Senior Lecturer, Surgeon, Department of Transplantation Surgery, Sheffield Teaching Hospitals, Herries Road, Sheffield S57AU, United Kingdom. ahmed.halawa@sth.nhs.uk
Telephone: +44-77-87542128 Fax: +44-11-42714604
Received: March 10, 2018
Peer-review started: March 11, 2018
First decision: March 30, 2018
Revised: June 26, 2018
Accepted: July 9, 2018
Article in press: July 10, 2018
Published online: September 10, 2018
Processing time: 181 Days and 0.2 Hours
Abstract

Thrombotic microangiopathy (TMA) is one of the most devastating sequalae of kidney transplantation. A number of published articles have covered either de novo or recurrent TMA in an isolated manner. We have, hereby, in this article endeavored to address both types of TMA in a comparative mode. We appreciate that de novo TMA is more common and its prognosis is poorer than recurrent TMA; the latter has a genetic background, with mutations that impact disease behavior and, consequently, allograft and patient survival. Post-transplant TMA can occur as a recurrence of the disease involving the native kidney or as de novo disease with no evidence of previous involvement before transplant. While atypical hemolytic uremic syndrome is a rare disease that results from complement dysregulation with alternative pathway overactivity, de novo TMA is a heterogenous set of various etiologies and constitutes the vast majority of post-transplant TMA cases. Management of both diseases varies from simple maneuvers, e.g., plasmapheresis, drug withdrawal or dose modification, to lifelong complement blockade, which is rather costly. Careful donor selection and proper recipient preparation, including complete genetic screening, would be a pragmatic approach. Novel therapies, e.g., purified products of the deficient genes, though promising in theory, are not yet of proven value.

Keywords: Kidney transplantation; De novo thrombotic microangiopathy; Thrombotic microangiopathy; Recurrent thrombotic microangiopathy; Atypical hemolytic uremic syndrome

Core tip: Many articles in the literature have covered either de novo or recurrent thrombotic microangiopathy (TMA) in an isolated manner; we tried here in this article to gather the criteria of both types in one review for comparison. Contrary to what was believed in the past, de novo TMA is more common and its prognosis is poorer. On the other hand, recurrent TMA relies on a wide base of genetic backgrounds, with mutation errors differing in their impact on disease behavior and consequently on allograft and patient survival. This base for instance is rapidly expanding, and ultimately warrants a parallel robust work up regimen.