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Prof. PhD C.J. Fijnvandraat

Position
Full Professor
Main activities
Education, Patient care, Research
Specialisation
Pediatric Hematology
Focus of research

Hemophilia

Sickle Cell Disease

 

Key publications
  • Eckhardt Corien L., van Velzen Alice S., Peters Marjolein, Astermark Jan, Brons Paul P., Castaman Giancarlo, Cnossen Marjon H., Dors Natasja, Escuriola-Ettingshausen Carmen, Hamulyak Karly, Hart Daniel P., Hay Charles R. M., Haya Saturnino, van Heerde Waander L., Hermans Cedric, Holmström Margareta, Jimenez-Yuste Victor, Keenan Russell D., Klamroth Robert, Laros-van Gorkom Britta A. P., Leebeek Frank W. G., Liesner Ri, Mäkipernaa Anne, Male Christoph, Mauser-Bunschoten Evelien, Mazzucconi Maria G., McRae Simon, Meijer Karina, Mitchell Michael, Morfini Massimo, Nijziel Marten, Oldenburg Johannes, Peerlinck Kathelijne, Petrini Pia, Platokouki Helena, Reitter-Pfoertner Sylvia E., Santagostino Elena, Schinco Piercarla, Smiers Frans J., Siegmund Berthold, Tagliaferri Annarita, Yee Thynn T., Kamphuisen Pieter Willem, van der Bom Johanna G., Fijnvandraat Karin Factor VIII gene (F8) mutation and risk of inhibitor development in nonsevere hemophilia A Blood 2013;122 (11):1954-1962 [PubMed]
  • Castaman Giancarlo, Fijnvandraat Karin Molecular and clinical predictors of inhibitor risk and its prevention and treatment in mild hemophilia A Blood 2014;124 (15):2333-2336 [PubMed]
  • Fijnvandraat Karin, Cnossen Marjon H., Leebeek Frank W. G., Peters Marjolein Diagnosis and management of haemophilia BMJ (Clinical research ed.) 2012;344:e2707 [PubMed]
  • van Velzen Alice S., Eckhardt Corien L., Streefkerk Nina, Peters Marjolein, Hart Daniel P., Hamulyak Karly, Klamroth Robert, Meijer Karina, Nijziel Marten, Schinco Piercarla, Yee Thynn T., van der Bom Johanna G., Fijnvandraat Karin, Kamphuisen P. W., van der Bom J. G., Peerlinck K., Oldenburg J., Hay C. R. M., Santagostino E., Astermark J., Loomans J. L., van Eijkelenburg A., Jansen A. J., Kruijt C. C., van Tienoven B., van Baar A. C. G., Corten I. W., Bultje A. C., Visser G. R., Meijer K., Nijziel M., Dors N., Hamulyak K., Brons P. P., Laros-van Gorkom B. A. P., van Heerde W. L., Leebeek F. W. G., Kruip M., Cnossen M. H., Mauser-Bunschoten E. P., Fischer K., Smiers F. J., Hermans C., Schwaab R., Siegmund B., Klamroth R., Escuriola-Ettingshausen C., Königs C., Petrini P., Holmström M. The incidence and treatment of bleeding episodes in non-severe haemophilia A patients with inhibitors Thrombosis and haemostasis 2016;115 (3):543-550 [PubMed]
  • Eckhardt C. L., Loomans J. I., van Velzen A. S., Peters M., Mauser-Bunschoten E. P., Schwaab R., Mazzucconi M. G., Tagliaferri A., Siegmund B., Reitter-Pfoertner S. E., van der Bom J. G., Fijnvandraat K., Kamphuisen P. W., Peerlinck K., Oldenburg J., Santagostino E., Astermark J., Streefkerk N., Loomans J. L., van Eijkelenburg A., Jansen A. J., Kruijt C. C., van Tienoven B., van Baar A. C. G., Corten I. W., Meijer K., Nijziel M. R., Dors N., Hamulyak K., Beckers E., Brons P. P., Laros-van Gorkom B. A. P., van Heerde W. L., Leebeek F., Kruip M., Cnossen M. H., Mauser-Bunschoten E., Fischer K., Smiers F. J., Hermans C., Klamroth R., Escuriola-Ettingshausen C., Königs C., Petrini P., Holmström M., Mäkipernaa A., Male C., Pabinger I., Keenan R. D., Liesner R. Inhibitor development and mortality in non-severe hemophilia A Journal of thrombosis and haemostasis 2015;13 (7):1217-1225 [PubMed]
All Publications
Research programmes

Prof. PhD C.J. Fijnvandraat (Etiology of inhibitor development in hemophilia & Alloimunisation in Sickle Cell Disease)

Etiology of inhibitor development in hemophilia

Hemophilia A (Factor VIII deficiency) is a rare bleeding disorder that is treated by intravenous administration of clotting factor concentrates. A major challenge in the treatment is the development of inhibiting antibodies (inhibitors), directed towards F VIII. Inhibitors block the action of F VIII and thereby increase morbidity and mortality.

This research line aims to identify clinical and genetic risk factors for inhibitor development, especially in mild/moderate hemophilia.

  • The INSIGHT study is an international cohort study performed in 34 Hemophilia Treatment Centers in 11 countries that has included 2700 moderate and mild hemophilia A patients. Genetic and clinical risk factors for inhibitor development will be identified in this cohort.
  • Treatment of inhibitors in moderate and mild hemophilia is investigated in the TRIM study.
  • DDAVP can be used as a treatment for bleedings circumventing the use of clotting factor concentrates, thereby reducing the risk of inhibitor development. The RISE study identifies predictors of DDAVP response in mild hemophilia A patients.
  • Small changes in immunological responses may identify patients that will develop an inhibitor in an early stage. The PUPS study aims to analyze the immunological response in young children after their first exposures to F VIII concentrate.

 

Cerebral infarction and vaso-occlusion in Sickle Cell Disease


Sickle Cell Disease is a hereditary anemia caused by a mutation in the beta-globulin gene, that occurs predominantly in persons with African ancestry. Hallmarks of the disease are chronic haemolytic anemia and vascular occlusion, that may cause irreversible damage to all vital organs.


This research line aims to identify risk factors for “silent” cerebral infarction and to identify and treat its neuropsychological sequelae. We also study the pathophysiological processes of (painfull) vaso-occlusive crises and interventions to prevent these.
• FIND study: Finding Indicators of Neurological Damage is a multicenter observational cohort study that aims to identify genetic and clinical risk factors for “silent” cerebral infarction
• KLIK study: Quality of Life in Clinical Practice identifies (neuropsychological) problems in children with Sickle Cell Disease. By early identification therapeutic measures can be initiated as early as possible
• STAR is a multicenter observational study aiming to unravel allo-antibody formation in patients with Sickle Cell Disease
 

Faculty
PhD M. Peters

Postdocs
MD PhD C.L. de Groot-Eckhardt
MD PhD S.C. Gouw

Prof. MD PhD T.W. Kuijpers (Infection and inflammation: on the crossroad of hematology and immunology)

Prof. PhD R.A.A. Mathot (Population pharmacokinetic-pharmacodynamic (PK-PD) research)

Current research funding
  • AMC
  • BAYER B.V.
  • Baxalta Innovations GmbH
  • Baxalta US Inc., a Delaware corporation
  • Bristol Myers Squibb
  • CSL Behring BV
  • CSL Behring GmbH
  • EAHAD
  • Erasmus MC
  • Global Blood Therapeutics
  • NIEUWE DEB IND
  • Novo Nordisk B.V.
  • Pfizer B.V. (4726)
  • Pfizer Inc. GFSS - Americas
  • Sanquin Blood Supply Foundation
  • Stichting tot Steun EKZ-AMC
  • Swedish Orphan Biovitrum BVBA/SPRL
  • The Hospital for Sick Children