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1. Full Text Hereditary Thrombotic Thrombocytopenic Purpura
by Kremer Hovinga, Johanna A and George, James N
The New England Journal of Medicine, ISSN 0028-4793, 10/2019, Volume 381, Issue 17, pp. 1653 - 1662
Hereditary thrombotic thrombocytopenic purpura is an autosomal recessive disorder caused by the absence of a functional protease (ADAMTS13) that processes von... 
TTP | MEDICINE, GENERAL & INTERNAL | PLASMA | POLYMORPHISMS | FACTOR-VIII | PURIFICATION | ADAMTS13 DEFICIENCY | VON-WILLEBRAND-FACTOR | FACTOR-CLEAVING PROTEASE | UPSHAW-SCHULMAN SYNDROME | PREGNANCY
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2. Full Text The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017
by van Dorland, HA and Taleghani, MM and Sakai, K and Friedman, KD and George, JN and Hrachovinova, I and Knobl, PN and von Krogh, AS and Schneppenheim, R and Aebi-Huber, I and Butikofer, L and Largiader, CR and Cermakova, Z and Kokame, K and Miyata, T and Yagi, H and Terrell, DR and Vesely, SK and Matsumoto, M and Lammle, B and Fujimura, Y and Hovinga, JAK and Hereditary TTP Registry
HAEMATOLOGICA, ISSN 0390-6078, 09/2019, Volume 104, Issue 10, pp. 2107 - 2115
Congenital thrombotic thrombocytopenic purpura is an autosomal recessive inherited disease with a clinically heterogeneous course and an incompletely... 
PATHOGENESIS | DIAGNOSIS | MANAGEMENT | PLASMA | UPSHAW-SCHULMAN-SYNDROME | ADAMTS13 MUTATIONS | PREVALENCE | HEMATOLOGY | ASSOCIATION | FACTOR-CLEAVING PROTEASE | PREGNANCY
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3. Full Text Acquired Hemophilia and Plasma Cell Neoplasms, a Rare Association
by Jalowiec, Katarzyna Aleksandra and Andres, Martin and Mansouri Taleghani, Behrouz and Angelillo-Scherrer, Anne and Rovo, Alicia and Kremer Hovinga, Johanna A
Blood, ISSN 0006-4971, 11/2019, Volume 134, Issue Supplement_1, pp. 4930 - 4930
Acquired hemophilia A (AHA) is a rare autoimmune disease caused by circulating autoantibodies inhibiting coagulation factor VIII (FVIII), leading to a... 
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4. Full Text Safety of Caplacizumab in Patients Without Documented Severe ADAMTS13 Deficiency During the HERCULES Study
by De La Rubia, Javier and Peyvandi, Flora and Scully, Marie and Cataland, Spero and Coppo, Paul and Kremer Hovinga, Johanna A and Knoebl, Paul and Metjian, Ara and Pavenski, Katerina and De Winter, Hilde and de Passos Sousa, Rui and Callewaert, Filip
Blood, ISSN 0006-4971, 11/2019, Volume 134, Issue Supplement_1, pp. 1093 - 1093
Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy caused by a deficiency in the... 
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5. Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura
by Kremer Hovinga, Johanna A and Lämmle, Bernhard
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program, ISSN 1520-4391, 2012, Volume 2012, pp. 610 - 616
The regulation of VWF multimer size is essential in preventing spontaneous microvascular platelet clumping, a central pathophysiologic finding in thrombotic... 
AUTOANTIBODIES | SPACER DOMAIN | PLASMA-EXCHANGE | HEMOLYTIC-UREMIC SYNDROME | FACTOR MULTIMERS | ANTI-ADAMTS13 ANTIBODIES | BINDING-SITE | HEMATOLOGY | VON-WILLEBRAND-FACTOR | EDUCATION, SCIENTIFIC DISCIPLINES | FACTOR-CLEAVING PROTEASE | UPSHAW-SCHULMAN SYNDROME | ADAM Proteins - physiology | Autoantibodies - chemistry | Humans | Middle Aged | Recombinant Proteins - chemistry | Male | Antigens - metabolism | Purpura, Thrombotic Thrombocytopenic - diagnosis | ADAMTS13 Protein | Homozygote | Endothelial Cells - cytology | Hematology - methods | Adult | Female | Heterozygote | Aged | Epitopes - chemistry | Purpura, Thrombotic Thrombocytopenic - therapy | Mutation | ADAM Proteins - genetics | Purpura, Thrombotic Thrombocytopenic - blood | Immunoglobulin G - metabolism | von Willebrand Factor - metabolism
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6. Full Text Description of the First Cases with ADAMTS13 Mutations in Hungary
by Réti, Marienn and Sinkovits, György and Cseprekál, Orsolya and Csuka, Dorottya and Szilágyi, Ágnes and Farkas, Zita and Klucsik, Zsolt and Szederjesi, Attila and Wágner, László and Reusz, György and Kremer Hovinga, Johanna A and Rigó, János and Masszi, Tamas and Prohászka, Zoltan
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 5003 - 5003
Abstract INTRODUCTION: Low ADAMTS13 activity is associated with an increased risk of thrombotic thrombocytopenic purpura (TTP). This may be caused by the... 
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7. Full Text Narratives of Patients with Fatal Outcomes During the Phase 2 TITAN and Phase 3 HERCULES Studies
by Cataland, Spero and Scully, Marie and Peyvandi, Flora and Knoebl, Paul and Kremer Hovinga, Johanna A and Coppo, Paul and Metjian, Ara and De La Rubia, Javier and Pavenski, Katerina and De Winter, Hilde and Callewaert, Filip
Blood, ISSN 0006-4971, 11/2019, Volume 134, Issue Supplement_1, pp. 4908 - 4908
Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare but life-threatening thrombotic microangiopathy, with an untreated mortality rate of... 
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8. Full Text Obinutuzumab in two patients suffering from immune‐mediated thrombotic thrombocytopenic purpura intolerant to rituximab
by Robertz, Judith and Andres, Martin and Mansouri Taleghani, Behrouz and Koneth, Irene and Binet, Isabelle and Kremer Hovinga, Johanna A
American Journal of Hematology, ISSN 0361-8609, 10/2019, Volume 94, Issue 10, pp. E259 - E261
HEMATOLOGY
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9. Full Text Efficacy of Caplacizumab in Patients with aTTP in the HERCULES Study According to Initial Immunosuppression Regimen
by Pavenski, Katerina and Knoebl, Paul and Scully, Marie and Kremer Hovinga, Johanna A and Coppo, Paul and Peyvandi, Flora and Cataland, Spero and Metjian, Ara and De La Rubia, Javier and De Winter, Hilde and de Passos Sousa, Rui and Callewaert, Filip
Blood, ISSN 0006-4971, 11/2019, Volume 134, Issue Supplement_1, pp. 2365 - 2365
Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is an acute, life-threatening thrombotic microangiopathy that requires urgent and specialized... 
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10. Full Text Efficacy of Caplacizumab in Patients with aTTP in the HERCULES Study According to Baseline Disease Severity
by Coppo, Paul and Cataland, Spero and Peyvandi, Flora and Knoebl, Paul and Scully, Marie and Kremer Hovinga, Johanna A and Metjian, Ara and De La Rubia, Javier and Pavenski, Katerina and De Winter, Hilde and de Passos Sousa, Rui and Callewaert, Filip
Blood, ISSN 0006-4971, 11/2019, Volume 134, Issue Supplement_1, pp. 2366 - 2366
Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy that involves abnormal... 
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11. Full Text Detection and Differential Diagnosis of Prekallikrein Deficiency: Genetic Study of New Families and Systematic Review of the Literature
by Barco, Stefano and Sollfrank, Stefanie and Trinchero, Alice and Tomao, Luigi and Zieger, Barbara and Kremer Hovinga, Johanna A and Conti, Laura and Adenäuer, Anke and Miloni, Erwin and Lackner, Karl J and Rossmann, Heidi and Lammle, Bernhard
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 2496 - 2496
Abstract Introduction. Prekallikrein (PK) and high-molecular-weight kininogen (HK) deficiencies are ultra-rare, autosomal-recessive defects of the contact... 
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12. Full Text Efficacy and safety of open‐label caplacizumab in patients with exacerbations of acquired thrombotic thrombocytopenic purpura in the HERCULES study
by Knoebl, Paul and Cataland, Spero and Peyvandi, Flora and Coppo, Paul and Scully, Marie and Kremer Hovinga, Johanna A and Metjian, Ara and de la Rubia, Javier and Pavenski, Katerina and Minkue Mi Edou, Jessica and De Winter, Hilde and Callewaert, Filip
Journal of Thrombosis and Haemostasis, ISSN 1538-7933, 11/2019
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13. Full Text Cellular Factor XIII, a Transglutaminase in Human Corneal Keratocytes
by Orosz, Zsuzsanna Z and Bárdos, Helga and Shemirani, Amir H and Beke Debreceni, Ildikó and Lassila, Riitta and Riikonen, Antti S and Kremer Hovinga, Johanna A and Seiler, Theo G and van Dorland, Hendrika A and Schroeder, Verena and Boda, Zoltán and Nemes, László and Früh Eppstein, Beatrice and Nagy, Bence and Facskó, Andrea and Kappelmayer, János and Muszbek, László
International Journal of Molecular Sciences, ISSN 1422-0067, 11/2019, Volume 20, Issue 23, p. 5963
Cellular factor XIII (cFXIII, FXIII-A2), a transglutaminase, has been demonstrated in a few cell types. Its main function is to cross-link proteins by... 
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14. Full Text Integrated Efficacy Results from the Phase II and Phase III Studies with Caplacizumab in Patients with Acquired Thrombotic Thrombocytopenic Purpura
by Peyvandi, Flora and Cataland, Spero R and Scully, Marie and Coppo, Paul and Knöbl, Paul and Kremer Hovinga, Johanna A and Metjian, Ara and De La Rubia, Javier and Pavenski, Katerina and Minkue, Jessica and Callewaert, Filip and De Winter, Hilde
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 373 - 373
Abstract Introduction : Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening thrombotic microangiopathy, in which accumulation of... 
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15. Full Text Integrated Safety Results from the Phase II and Phase III Studies with Caplacizumab in Patients with Acquired Thrombotic Thrombocytopenic Purpura
by Knöbl, Paul and Scully, Marie and Cataland, Spero R and Peyvandi, Flora and Coppo, Paul and Kremer Hovinga, Johanna A and Metjian, Ara and De La Rubia, Javier and Pavenski, Katerina and Minkue, Jessica and Callewaert, Filip and De Winter, Hilde
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 3739 - 3739
Abstract Introduction : During the clinical development of caplacizumab, safety and tolerability data have been accrued from Phase I, Phase II, and Phase III... 
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16. Full Text Risk Factors and Manageability of the Mainly Mild Mucocutaneous Bleeding Profile Observed in Attp Patients Treated with Caplacizumab during the Phase III Hercules Study
by Cataland, Spero R and Scully, Marie and Peyvandi, Flora and Coppo, Paul and Knöbl, Paul and Kremer Hovinga, Johanna A and Metjian, Ara and De La Rubia, Javier and Pavenski, Katerina and Minkue, Jessica and Sousa, Rui Passos and Callewaert, Filip and De Winter, Hilde
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 1142 - 1142
Abstract Introduction : Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune blood clotting disorder, manifested by... 
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17. Full Text Estimation of Nuwiq® (simoctocog alfa) activity using one‐stage and chromogenic assays—Results from an international comparative field study
by Tiefenbacher, Stefan and Albisetti, Manuela and Baker, Peter and Kappert, Guenther and Kitchen, Steve and Kremer Hovinga, Johanna A and Pouplard, Claire and Scholz, Ute and Ternisien, Catherine and Borgvall, Carin and Vicente, Tiago and Belyanskaya, Larisa and Walter, Olaf and Oldenburg, Johannes and Nuwiq Field Study Participating and Nuwiq Field Study Participating Laboratories and the Nuwiq Field Study Participating Laboratories
Haemophilia, ISSN 1351-8216, 07/2019, Volume 25, Issue 4, pp. 708 - 717
Background Accurate determination of coagulation factor VIII activity (FVIII:C) is essential for effective and safe FVIII replacement therapy. FVIII:C can be... 
coagulation factor VIII | field study | one‐stage assay | Nuwiq | chromogenic assay | human‐cl rhFVIII | EFFICACY | FACTOR-VIII | AFSTYLA | Nuwiq (R) | FULL-LENGTH | HUMAN CELL-LINE | VARIABILITY | HEMOPHILIC PLASMA | one-stage assay | SAMPLES | FVIII | HEMATOLOGY | human-cl rhFVIII | Comparative analysis | Cells | Blood coagulation factor VIII | Original
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18. Progress in Haemostasis
by Hovinga, JAK and Scharf, RE
HAMOSTASEOLOGIE, ISSN 0720-9355, 01/2017, Volume 37, Issue 1, pp. 9 - 11
HEMATOLOGY
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19. Full Text Safety of Caplacizumab for the Treatment of Patients with Acquired Thrombotic Thrombocytopenic Purpura - Results Normalized to Time of Exposure in a Double-Blind, Placebo-Controlled, Phase 3 Hercules Study
by Kremer Hovinga, Johanna A and Scully, Marie and Cataland, Spero R and Peyvandi, Flora and Coppo, Paul and Knöbl, Paul and De La Rubia, Javier and Pavenski, Katerina and Minkue, Jessica and Sousa, Rui Passos and Callewaert, Filip and De Winter, Hilde and Metjian, Ara
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 3744 - 3744
Abstract Introduction : Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening thrombotic microangiopathy. A severe deficiency in... 
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20. Progress in Haemostasis. From individual patients to pathophysiological insights
by Kremer Hovinga, Johanna A and Scharf, Rüdiger E
Hamostaseologie, ISSN 0720-9355, 01/2017, Volume 37, Issue 1, p. 9
Hemostasis | Humans | Hemostatic Techniques - trends | Blood Coagulation Disorders - diagnosis | Hematology - trends | Thromboembolism - diagnostic imaging | Germany | Thromboembolism - therapy | Blood Coagulation Disorders - therapy
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