Premium
Ciclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long‐term follow‐up with serial analysis of ADAMTS13 activity
Author(s) -
Cataland Spero R.,
Jin Ming,
Lin Shili,
Kennedy Melanie S.,
Kraut Eric H.,
George James N.,
Wu Haifeng M.
Publication year - 2007
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2007.06819.x
Subject(s) - adamts13 , medicine , thrombotic thrombocytopenic purpura , exacerbation , ciclosporin , context (archaeology) , gastroenterology , immunology , platelet , chemotherapy , paleontology , biology
Summary We hypothesized that ciclosporin (CSA) as adjunct to plasma exchange (PE) improves the efficacy of PE in idiopathic thrombotic thrombocytopenic purpura (TTP) via suppression of the antibody inhibitor of ADAMTS13. Our preliminary findings with CSA and PE as the upfront treatment of TTP suggested that the addition of CSA to PE significantly decreased the exacerbation (disease recurrence within 30 d of the last PE) rates compared to a cohort that received corticosteroids and PE as their upfront therapy of TTP. We present an updated analysis with long‐term follow‐up of 18 patients with idiopathic TTP treated with concurrent CSA and PE with analysis of serial measurements of ADAMTS13 activity, antigen and inhibitor concentration in the context of clinical outcome data. Overall, 16/18 (89%) patients achieved remission, similar to historical remission rates in idiopathic TTP with PE with only one patient suffering an exacerbation. Clinical responses correlated with improvements in ADAMTS13 activity and suppression of the antibody inhibitor of ADAMTS13. These data suggest that the efficacy of CSA is at least in part related to its suppression of the antibody inhibitor of ADAMTS13 and a subsequent improvement in ADAMTS13 activity and antigen.