Complement and cytokine response in acute Thrombotic Thrombocytopenic Purpura

• Published in: British journal of haematology 2014-03, Vol.164 (6), p.858-866
• Main Authors: Westwood, John‐Paul, Langley, Kathryn, Heelas, Edward, Machin, Samuel J., Scully, Marie
• Format: Article
• Language: English
• Subjects: ADAM Proteins - immunology; ADAMTS13; Adolescent; Adult; Aged; Atypical Hemolytic Uremic Syndrome; Biological and medical sciences; Cohort Studies; complement; Complement Activation - immunology; cytokines; Cytokines - immunology; Female; Hematologic and hematopoietic diseases; Hemolytic-Uremic Syndrome - immunology; Hemolytic-Uremic Syndrome - pathology; Humans; Male; Medical sciences; Middle Aged; Platelet diseases and coagulopathies; Platelets, Haemostasis and Thrombosis; Prospective Studies; Purpura, Thrombotic Thrombocytopenic - immunology; Purpura, Thrombotic Thrombocytopenic - pathology; T cells; Thrombotic Thrombocytopenic Purpura; Tumors; Young Adult
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/bjh.12707

Summary Complement dysregulation is key in the pathogenesis of atypical Haemolytic Uraemic Syndrome (aHUS), but no clear role for complement has been identified in Thrombotic Thrombocytopenic Purpura (TTP). We aimed to assess complement activation and cytokine response in acute antibody‐mediated TTP. Complement C3a and C5a and cytokines (interleukin (IL)‐2, IL‐4, IL‐6, IL‐10, tumour necrosis factor, interferon‐γ and IL‐17a) were measured in 20 acute TTP patients and 49 remission cases. Anti‐ADAMTS13 immunoglobulin G (IgG) subtypes were measured in acute patients in order to study the association with complement activation. In acute TTP, median C3a and C5a were significantly elevated compared to remission, C3a 63·9 ng/ml vs. 38·2 ng/ml (P