A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura
A previously healthy 35-year-old woman presented with severe thrombotic thrombocytopenic purpura (TTP) affecting several organs and concomitant influenza A infection. On admission to hospital, haemoglobin was 5.4▊g/dl, platelet count 6▊×▊109/l,
Schistocyte count in peripheral blood 5%, and throat swab positive for influenza A RNA. The patient was treated with antiviral medication and transfusions of fresh frozen plasma before plasma exchange therapy with excellent response. Plasmaphereses were attenuated after 5 days, resulting in
TTP relapse 3 days later. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) protein was very low (<0.04â–ŠU/l) and anti-ADAMTS13 elevated (>2â–ŠU/l), consistent with acquired TTP. Platelet counts normalized
after five additional plasma exchanges and oral corticosteroids. Antinuclear antibodies and subgroup anti-Ro/Sjögren's syndrome A antigen (SSA) were detected in serum and have remained borderline-elevated, although evaluation during TTP, recovery and follow-up have lacked clinical
manifestations of connective tissue disease. Influenza A infection induced production of ADAMTS13 inhibitor, which resulted in TTP in a patient with circulating antinuclear antibodies, lacking other manifestations of connective tissue disease.
Keywords: influenza A; plasmapheresis; thrombotic thrombocytopenic purpura
Document Type: Research Article
Affiliations: 1: Department of Rheumatology 2: Division for Infectious Diseases, Department of Medicine 3: Division for Hematology, Department of Medicine, Haukeland University Hospital, Bergen, Institute of Medicine, University of Bergen, Bergen, Norway
Publication date: 01 June 2015
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