REIPERT, B. M., B. GANGADHARAN, C. J. HOFBAUER, V. BERG, H. SCHWEIGER, J. BOWEN, Jan BLATNÝ, K. FIJNVANDRAAT, E. S. MULLINS, J. KLINTMAN, C. MALE, C. MCGUINN, S. L. MEEKS, V. C. RADULESCU, M. V. RAGNI, M. RECHT, A. D. SHAPIRO, J. M. STABER, H. M. YAISH, E. SANTAGOSTINO a D. L. BROWN. The prospective Hemophilia Inhibitor PUP Study reveals distinct antibody signatures prior to FVIII inhibitor development. BLOOD ADVANCES. WASHINGTON: AMER SOC HEMATOLOGY, 2020, roč. 4, č. 22, s. 5785-5796. ISSN 2473-9529. Dostupné z: https://dx.doi.org/10.1182/bloodadvances.2020002731. |
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@article{1726136, author = {Reipert, B. M. and Gangadharan, B. and Hofbauer, C. J. and Berg, V. and Schweiger, H. and Bowen, J. and Blatný, Jan and Fijnvandraat, K. and Mullins, E. S. and Klintman, J. and Male, C. and McGuinn, C. and Meeks, S. L. and Radulescu, V. C. and Ragni, M. V. and Recht, M. and Shapiro, A. D. and Staber, J. M. and Yaish, H. M. and Santagostino, E. and Brown, D. L.}, article_location = {WASHINGTON}, article_number = {22}, doi = {http://dx.doi.org/10.1182/bloodadvances.2020002731}, keywords = {Hemophilia Inhibitor PUP; FVIII inhibitor development}, language = {eng}, issn = {2473-9529}, journal = {BLOOD ADVANCES}, title = {The prospective Hemophilia Inhibitor PUP Study reveals distinct antibody signatures prior to FVIII inhibitor development}, url = {https://watermark.silverchair.com/advancesadv2020002731.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAABA4wggQKBgkqhkiG9w0BBwagggP7MIID9wIBADCCA_AGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQM4i795NteaBYi_4jtAgEQgIIDwbi72fPiY61518P6v9IR00njkDl3nDKG}, volume = {4}, year = {2020} }
TY - JOUR ID - 1726136 AU - Reipert, B. M. - Gangadharan, B. - Hofbauer, C. J. - Berg, V. - Schweiger, H. - Bowen, J. - Blatný, Jan - Fijnvandraat, K. - Mullins, E. S. - Klintman, J. - Male, C. - McGuinn, C. - Meeks, S. L. - Radulescu, V. C. - Ragni, M. V. - Recht, M. - Shapiro, A. D. - Staber, J. M. - Yaish, H. M. - Santagostino, E. - Brown, D. L. PY - 2020 TI - The prospective Hemophilia Inhibitor PUP Study reveals distinct antibody signatures prior to FVIII inhibitor development JF - BLOOD ADVANCES VL - 4 IS - 22 SP - 5785-5796 EP - 5785-5796 PB - AMER SOC HEMATOLOGY SN - 24739529 KW - Hemophilia Inhibitor PUP KW - FVIII inhibitor development UR - https://watermark.silverchair.com/advancesadv2020002731.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAABA4wggQKBgkqhkiG9w0BBwagggP7MIID9wIBADCCA_AGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQM4i795NteaBYi_4jtAgEQgIIDwbi72fPiY61518P6v9IR00njkDl3nDKG L2 - https://watermark.silverchair.com/advancesadv2020002731.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAABA4wggQKBgkqhkiG9w0BBwagggP7MIID9wIBADCCA_AGCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQM4i795NteaBYi_4jtAgEQgIIDwbi72fPiY61518P6v9IR00njkDl3nDKG N2 - Preventing factor VIII (FVIII) inhibitors following replacement therapies with FVIII products in patients with hemophilia A remains an unmet medical need. Better understanding of the early events of evolving FVIII inhibitors is essential for risk identification and the design of novel strategies to prevent inhibitor development. The Hemophilia Inhibitor Previously Untreated Patients (PUPs) Study (HIPS; www. clinicaltrials.gov #NCT01652027) is the first prospective cohort study to evaluate comprehensive changes in the immune system during the first 50 exposure days ( EDs) to FVIII in patients with severe hemophilia A. HIPS participants were enrolled prior to their first exposure to FVIII or blood products ("true PUPs") and were evaluated for different immunological and clinical parameters at specified time points during their first 50 EDs to a single source of recombinant FVIII. Longitudinal antibody data resulting from this study indicate that there are 4 subgroups of patients expressing distinct signatures of FVIII- binding antibodies. Subgroup 1 did not develop any detectable FVIII-binding immunoglobulin G (IgG) antibodies. Subgroup 2 developed nonneutralizing, FVIII-binding IgG1 antibodies, but other FVIII-binding IgG subclasses were not observed. Subgroup 3 developed transient FVIII inhibitors associated with FVIII-binding IgG1 antibodies, similar to subgroup 2. Subgroup 4 developed persistent FVIII inhibitors associated with an initial development of high-affinity, FVIII-binding IgG1 antibodies, followed by IgG3 and IgG4 antibodies. Appearance of FVIII-binding IgG3 was always associated with persistent FVIII inhibitors and the subsequent development of FVIII-binding IgG4. Some of the antibody signatures identified in HIPS could serve as candidates for early biomarkers of FVIII inhibitor development. ER -
REIPERT, B. M., B. GANGADHARAN, C. J. HOFBAUER, V. BERG, H. SCHWEIGER, J. BOWEN, Jan BLATNÝ, K. FIJNVANDRAAT, E. S. MULLINS, J. KLINTMAN, C. MALE, C. MCGUINN, S. L. MEEKS, V. C. RADULESCU, M. V. RAGNI, M. RECHT, A. D. SHAPIRO, J. M. STABER, H. M. YAISH, E. SANTAGOSTINO a D. L. BROWN. The prospective Hemophilia Inhibitor PUP Study reveals distinct antibody signatures prior to FVIII inhibitor development. \textit{BLOOD ADVANCES}. WASHINGTON: AMER SOC HEMATOLOGY, 2020, roč.~4, č.~22, s.~5785-5796. ISSN~2473-9529. Dostupné z: https://dx.doi.org/10.1182/bloodadvances.2020002731.
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