Secondary thrombotic microangiopathy with severely reduced ADAMTS13 activity in a patient with Capnocytophaga canimorsus sepsis: a case report. Issue 10 (17th September 2018)

• Record Type: Journal Article
• Authors: Smeets, Nori J.L.; Fijnheer, Rob; Sebastian, Silvie; De Mast, Quirijn
• Is Part Of: Transfusion. Volume 58:Issue 10(2018)
• Page Start: 2426
• Page End: 2429
• Publication Date: 2018-09-17
• Subjects: Hematology -- Periodicals; Blood -- Transfusion -- Periodicals; Blood Group Antigens -- Periodicals; Blood Preservation -- Periodicals; Blood Transfusion -- Periodicals; 615
• DOI: 10.1111/trf.14829
• Languages: English
• ISSNs: 0041-1132

Abstract

Abstract : BACKGROUND: The Gram‐negative bacillus Capnocytophaga canimorsus may cause a severe illness resembling thrombotic thrombocytopenic purpura (TTP). The pathogenesis and optimal therapy of this secondary thrombotic microangiopathy (TMA) remain uncertain. CASE REPORT: A 63‐year‐old Caucasian man was admitted with suspicion for TTP, but blood cultures grew C. canimorsus . Initial investigations revealed severe thrombocytopenia, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity level of less than 1%, and strongly elevated D‐dimer and lactate dehydrogenase levels. He made a full recovery with antibiotics and plasma infusion for 3 days. Plasmapheresis was not performed. Retrospective determination of serial ADAMTS13 activity levels revealed that ADAMTS13 activity had already increased to 25% at the start of plasma infusion. CONCLUSION: This case highlights that a C. canimorsus sepsis may cause a secondary TMA with a severe ADAMTS13 deficiency. It also illustrates that the adjunctive role of plasma exchange or plasma infusion is doubtful as ADAMTS13 activity levels increased with antibiotics alone.