{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T04:55:21Z","timestamp":1781758521821,"version":"3.54.5"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Thrombotic microangiopathies (TMAs) encompass a spectrum of severe pathological conditions mostly characterized by hemolytic anemia, microvascular thrombosis with organ failure, and thrombocytopenia. The etiological spectrum of TMA is diverse, with a notable association in transplant recipients, particularly linked to the administration of calcineurin inhibitors (CNI) and due to perioperative stress factors. The clinical case presented concerns the occurrence of complement-mediated TMA (cTMA) in a recipient following liver transplantation (LT) who had previously been diagnosed with autoimmune hepatitis (AIH). The patient received total plasma exchange, followed by treatment with the anti-complement factor C5 antibody ravulizumab shortly after diagnosis. Significant improvement in the patient\u2019s clinical condition and a decline in renal impairment was observed. Subsequently, dialysis therapy could be discontinued. In addition, an enhancement in liver functionality was detected. The diagnosis of cTMA was undoubtedly attributable to a multifactorial cause. Genetic testing identified variants involving complement factor H-related protein 5 (CFHR5) and complement component 6 (C6). The identified C6 variant was classified as a variant of uncertain significance (VUS), and its pathogenic relevance in complement-mediated TMA remains unclear. Notably, a sustained clinical response was documented six months after starting ravulizumab treatment, highlighting the complexities of managing complement-mediated disorders and the potential for durable responses using the therapy at the earliest possible time.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1774160","type":"journal-article","created":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T04:37:56Z","timestamp":1781757476000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Complement mediated thrombotic microangiopathy after liver transplantation in combination with a novel C6 variant of uncertain significance"],"prefix":"10.3389","volume":"17","author":[{"given":"Nicola Sariye","family":"Pollmann","sequence":"first","affiliation":[{"name":"Department of General, Visceral, and Vascular Surgery, Jena University Hospital","place":["Jena, 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