Hemolytic uremic syndrome and kidney transplantation in uncontrolled donation after circulatory death (DCD): A two-case report

Background: Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, throm-bocytopenia, and renal involvement. Com-plement-mediated atypical HUS (aHUS) is a result of genetic defects in the alternative complement pathway components or regu-lators. The introduction of eculizumab has improved renal and overall survival of aHUS patients. Nowadays, given organ shortage, it is necessary to consider kidney transplantation (KT) even in protocols with a high risk of HUS recurrence, such as from donation after circulatory death (DCD) donors. Here, we describe two patients with HUS who underwent a KT from an uncontrolled DCD (uDCD). Case summary: The first patient, affected by aHUS due to a heterozygous deletion in CFHR3-CFHR1 and a novel hetero-zygous variant in CFHR5 gene, underwent a KT with eculizumab prophylaxis. The patient did not experience a post-transplant aHUS recurrence. The second patient, who expe-rienced an HUS episode characterized by a hypertensive crisis and with no underlying mutations in complement system genes, underwent a KT without eculizumab prophy-laxis. At day 5, anti-complement treatment commenced due to hematological signs of thrombotic microangiopathy (TMA). After the introduction of eculizumab, we observed a stabilization of kidney function and hema-tological remission. Conclusion: We present herein two different patients with HUS who both underwent successful KT from uDCD donation under the umbrella of eculizumab therapy. Taking into account the importance of increasing the number of organs available for transplantation, uDCD could represent an additional resource in this subset of HUS pa-tients. © 2021 Dustri-Verlag Dr. K. Feistle.