ArticleFrontiers in medicine2026
Case Report: Complement-mediated thrombotic microangiopathy masquerading as a pancreatic mass.
Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Complement-mediated thrombotic microangiopathy (CM-TMA) results from dysregulated alternative pathway activation. Although extra-renal manifestations are well-recognized, presentation as a pancreatic mass is exceedingly rare and risks misdiagnosis as malignancy, delaying critical intervention. Case presentation: A 28-year-old male presented with malignant hypertension and rapidly progressive renal impairment. Abdominal CT revealed a pancreatic tail mass suspicious for neoplasia. However, histopathology of both renal and pancreatic tissues demonstrated microvascular thrombosis and endothelial injury, pointing to a systemic TMA process. Subsequent genetic testing confirmed pathogenic variants in CFH (p.Tyr1058His, p.Val1060Leu) and THBD (p.Asp486Tyr), establishing the diagnosis of CM-TMA. Intervention: Treatment was initiated with eculizumab alongside intensive renin-angiotensin system blockade. Outcomes: After 6 months of therapy, renal function recovered and the pancreatic mass completely resolved. However, 3 months after self-discontinuing eculizumab, the patient experienced a severe disease relapse and subsequently entered maintenance hemodialysis. Conclusion: This first histopathologically confirmed case of adult CM-TMA presenting as a pancreatic mass expands the known phenotype of complement-mediated disease. It highlights that in young adults with unexplained malignant hypertension and multisystem involvement, prompt evaluation for complement abnormalities is critical. The presence of high-risk complement variants and the relapse following treatment cessation underscore the necessity for long-term complement inhibition in such patients.
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