@article{3102423,
    title = "Real-world data of thrombotic microangiopathy management: The key role of ADAMTS13 activity and complement testing",
    author = "Gavriilaki, E. and Koravou, E.-E. and Chatziconstantinou, T. and Kalpadaki, C. and Printza, N. and Ximeri, M. and Christoforidou, A. and Karavalakis, G. and Kaliou, M. and Kalaitzidou, V. and Tassi, I. and Tzellou, M. and Touloumenidou, T. and Papalexandri, A. and Papathanasiou, M. and Syrigou, A. and Kioumi, A. and Liga, M. and Kaiafa, G. and Spyridonidis, A. and Kapsali, E. and Kollios, K. and Mandala, E. and Vlachaki, E. and Tsirigotis, P. and Papadaki, E. and Lalayanni, C. and Sakellari, I. and Anagnostopoulos, A.",
    journal = "Thrombosis Update",
    year = "2021",
    volume = "3",
    publisher = "Elsevier B.V.",
    doi = "10.1016/j.tru.2021.100043",
    abstract = "ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 motifs) activity is a key tool in differential diagnosis of thrombotic microangiopathies (TMAs). Due to the lack of availability of ADAMTS13 testing, PLASMIC/PLASIC scores have been suggested to predict ADAMTS13 deficiency. The importance of differentiating TTP from other complement-mediated TMAs is highlighted by the need to urgently start plasma exchange and utility of treatments such as caplacizumab or eculuzimab. Therefore, we aimed to evaluate ADAMTS13 activity, PLASMIC/PLASIC scores, and complement testing in guiding management of a real-world TMA cohort. We enrolled consecutive TMA patients with samples referred to our Center (01/2018–2020). If ADAMTS13 ​> ​10%, soluble C5b-9 was measured. Among 80 TMA patients, ADAMTS13 activity was ≤10% in 50 patients, while 28 had a relapsing disease. PLASMIC/PLASIC were excellent predictors of ADAMTS13 deficiency, especially in patients without secondary causes. Soluble C5b-9 levels were elevated (median 525 ​ng/ml, range 313–913 ​ng/ml) in 7 patients without secondary causes and ADAMTS13 ​> ​10% (hemolytic uremic syndrome/HUS). Two were shiga-toxin associated; while 5 atypical HUS. Only 1/5 patients received eculizumab and achieved TMA resolution implemented by guidance based on soluble C5b-9 levels. In transplant-associated TMA, 8/16 patients not responding to first-line treatment received eculizumab due to elevated C5b-9 levels (median 353 ​ng/ml, range 281–1252 ​ng/ml) and achieved TMA resolution. In conclusion, our real-world data confirm that ADAMTS13, complement testing, and PLASMIC/PLASIC are valuable tools in diagnosis and management of TMAs, but also highlight the unmet need of using available markers and treatments in clinical practice. © 2021"
}