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3 results on '"Chatziconstantinou T"'

1. Real-world data of thrombotic microangiopathy management: The key role of ADAMTS13 activity and complement testing

Author

Gavriilaki, E. Koravou, E.-E. Chatziconstantinou, T. Kalpadaki, C. Printza, N. Ximeri, M. Christoforidou, A. Karavalakis, G. Kaliou, M. Kalaitzidou, V. Tassi, I. Tzellou, M. Touloumenidou, T. Papalexandri, A. Papathanasiou, M. Syrigou, A. Kioumi, A. Liga, M. Kaiafa, G. Spyridonidis, A. Kapsali, E. Kollios, K. Mandala, E. Vlachaki, E. Tsirigotis, P. Papadaki, E. Lalayanni, C. Sakellari, I. Anagnostopoulos, A.

Subjects
hemic and lymphatic diseases
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

Published
2021

2. Caplacizumab for immune thrombotic thrombocytopenic purpura: real-world multicenter data.

Author

Gavriilaki E, Nikolousis E, Koravou EE, Dimou-Besikli S, Kartsios C, Papakonstantinou A, Mpanti A, Pontikoglou C, Kalpadaki C, Bitsani A, Tassi I, Touloumenidou T, Chatziconstantinou T, Papathanasiou M, Syrigou A, Ztriva E, Kaiafa G, Mandala E, Mellios Z, Karakasis D, Kourakli A, Symeonidis A, Kapsali E, Papadaki HH, Lalayanni C, and Sakellari I

Abstract

Given the limited real-world data of caplacizumab, our multicenter real-world study was designed to assess the safety and efficacy of caplacizumab in immune thrombotic thrombocytopenic pupura (iTTP), compared to historic controls. We have studied 70 patients: 23 in the caplacizumab and 47 in the historic control group. Plasma exchange was applied in all episodes except for two patients that denied plasma exchange. Rituximab as first-line treatment was more common in the caplacizumab group compared to historic control. Caplacizumab (10 mg daily) was given at a median on day 7 (1-43) from initial diagnosis for 32 (6-47) dosages. In the caplacizumab group, a median of 12 (8-23) patients required plasma exchange sessions versus 14 (6-32) in the control group. Caplacizumab administration did not produce any grade 3 complications or major hemorrhagic events. After a median of 19.0 (2.6-320) months since the iTTP diagnosis, 5 deaths occurred (4 in the control group and 1 in the caplacizumab group, p  = 0.310). Caplacizumab patients achieved early platelet normalization and ADAMTS13 activity normalization at the end of treatment. Relapse was observed only in 2/23 (9%) caplacizumab patients, compared to 29/47 (62%) historic controls ( p  < 0.001). Overall, caplacizumab is safe and effective in treating iTTP, including cases refractory to plasma exchange, re-administration, and cases without previous plasma exchange treatment. No major hemorrhagic events were observed. Cessation of dosing guided by ADAMTS13 has ensured a low relapse rate., Competing Interests: AntS and ArgS have received grants, honoraria or travel support from Abbvie, Amgen, Bei-Gene, BMS, Gilead, Glaxo, Janssen, MSD, Pfizer, Roche, Sanofi, Servier, SOBI, Takeda. ChaK and ChrK have received honoraria from Pfizer, BMS, Bayer, Takeda. EG and CP have received honoraria from Sanofi. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Gavriilaki, Nikolousis, Koravou, Dimou-Besikli, Kartsios, Papakonstantinou, Mpanti, Pontikoglou, Kalpadaki, Bitsani, Tassi, Touloumenidou, Chatziconstantinou, Papathanasiou, Syrigou, Ztriva, Kaiafa, Mandala, Mellios, Karakasis, Kourakli, Symeonidis, Kapsali, Papadaki, Lalayanni and Sakellari.)

Published
2023
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