Magnus Holter Bjørkto1, Andreas Barratt-Due2, Ingvild Nordøy3,4, Christina Dörje5, Eivind Galteland6, Andreas Lind7, Abdulkarim Hilli8, Pål Aukrust3,4, Geir Mjøen5. 1. Department of Transplant Medicine, Oslo University Hospital, Birch-Reichenwaldsgate 34, NO-0483, Oslo, Norway. mbjorkto@gmail.com. 2. Division of Critical care and Emergencies, Oslo University Hospital, Oslo, Norway. 3. Section for Clinical Immunology and Infectious Diseases, Oslo University Hospital, Oslo, Norway. 4. Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway. 5. Department of Transplant Medicine, Oslo University Hospital, Birch-Reichenwaldsgate 34, NO-0483, Oslo, Norway. 6. Department of Haematology, Oslo University Hospital, Oslo, Norway. 7. Department of Microbiology, Oslo University Hospital, Oslo, Norway. 8. Department of Internal Medicine, Diakonhjemmet Hospital, Oslo, Norway.
Abstract
BACKGROUND: The use of complement inhibition is well established for complement mediated thrombotic microangiopathy, but its role in secondary forms of thrombotic microangiopathy is debated. We here present a case of thrombotic microangiopathy triggered by Capnocytophaga canimorsus, illustrating the diagnostic difficulties in discriminating between different thrombotic microangiopathies, and the dilemmas regarding how to treat this disease entity. CASE PRESENTATION: A previously healthy 56-year-old woman presented with fever and confusion. She was diagnosed with sepsis from Capnocytophaga canimorsus and thrombotic microangiopathy. Marked activation of both T-cells, endothelium and complement were documented. She was successfully treated with antimicrobial therapy, the complement inhibitor eculizumab and splenectomy. After several weeks, a heterozygote variant in complement factor B was localized, potentially implying the diagnosis of a complement mediated TMA over an isolated infection related TMA. CONCLUSIONS: We discuss the possible interactions between complement activation and other findings in severe infection and argue that complement inhibition proved beneficial to this patient's rapid recovery.
BACKGROUND: The use of complement inhibition is well established for complement mediated thrombotic microangiopathy, but its role in secondary forms of thrombotic microangiopathy is debated. We here present a case of thrombotic microangiopathy triggered by Capnocytophaga canimorsus, illustrating the diagnostic difficulties in discriminating between different thrombotic microangiopathies, and the dilemmas regarding how to treat this disease entity. CASE PRESENTATION: A previously healthy 56-year-old woman presented with fever and confusion. She was diagnosed with sepsis from Capnocytophaga canimorsus and thrombotic microangiopathy. Marked activation of both T-cells, endothelium and complement were documented. She was successfully treated with antimicrobial therapy, the complement inhibitor eculizumab and splenectomy. After several weeks, a heterozygote variant in complement factor B was localized, potentially implying the diagnosis of a complement mediated TMA over an isolated infection related TMA. CONCLUSIONS: We discuss the possible interactions between complement activation and other findings in severe infection and argue that complement inhibition proved beneficial to this patient's rapid recovery.
Entities:
Keywords:
Capnocytophaga canimorsus; Case report; Complement; Eculizumab; Thrombotic microangiopathy
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