Frontiers in medicine · 2026
Case Report: Complement-mediated thrombotic microangiopathy masquerading as a pancreatic mass.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Sun Chuanchuan, Ding Ying, Yang Fan, Ding Yunfei, Qu Zhen, Yu Feng
Аннотация
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.
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.