title: A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura.
abstract: 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 × 10/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 (<0.04 U/l) and anti-ADAMTS13 elevated (>2 U/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.
authors: aDepartment of Rheumatology bDivision for Infectious Diseases, Department of Medicine cDivision for Hematology, Department of Medicine, Haukeland University Hospital, Bergen dInstitute of Medicine, University of Bergen, Bergen, Norway.
authors: Maria K
authors: MK
authors: Jonsson
authors: Daniel
authors: D
authors: Hammenfors
authors: Oddvar
authors: O
authors: Oppegaard
authors: Øystein
authors: Ø
authors: Bruserud
authors: Astrid Olsnes
authors: AO
authors: Kittang
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D000998:Antiviral Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D009980:Influenza A virus / Q000302:isolation & purification; D007251:Influenza, Human / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D010951:Plasma Exchange; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000150:complications* / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D000998:Antiviral Agents; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human