The Landsteiner Foundation for Blood Transfusion Research (LSBR) supports clinical and experimental scientific research in the field of blood, blood-forming tissue, blood products, and blood (related) diseases, provided that the research bears a relationship to the field of transfusion or transplantation of blood cells.
Mid August 2020 the book Surviving Haemophilia, the business case of a disease written by Cees Smit was published.
The book tells the story of Cees Smit, who was born with hemophilia A almost seventy years ago. No one ever expected him to survive long into adulthood, much less old age. He survived all stages of medical innovations, with both highlights and drawbacks. Today he is a lobbyist for patients’ rights and the improvement of patients’ position in the healthcare system. The book also serves as a strong plee for European self-sufficiency in ‘red’ and ‘white’ plasma from voluntary non-remunerated donors. It is disturbing that today’s policy makers pay so little attention to the world of blood transfusion and plasma products. In a neoliberal market economy, the trade in human substances is too easily accepted.
Publication was supported by a grant from the LSBR.
Submission for Pre-Applications for Scientific Research 2021 is now closed.
Granted projects 2020.
2005 – Dr Ruben Bierings – Hematology – ErasmusMC
New determinant of Weibel-Palade body shape and Von Willebrand factor trafficking within ER-Golgi SNARE networks.
The endothelium is a thin layer of endothelial cells (ECs) that forms the inner lining of blood vessels. Following damage to the vessel wall, the endothelium rapidly releases a pre-synthesized pool of hemostatic proteins which are stored in cigar-shaped organelles, Weibel-Palade bodies (WPBs). From these WPBs long strings of the hemostatic protein Von Willebrand factor (VWF) emerge, which are adhesive for platelets and which are crucial for platelet plug formation at the site of injury. This limits excessive blood loss following injury. Failure to release (sufficient amounts of) VWF leads to bleeding, such as in Von Willebrand disease (VWD) or in patients with “low VWF”. In this project we will study how VWF is trafficked by ECs, how ECs control the shape of WPBs and how this enables the endothelium to secrete sufficient amounts of VWF. This will help to explain why a large group of patients with clinical bleeding problems have reduced levels of VWF in their circulation.
2007 – Dr Micha Nethe – Hematology – Sanquin
E-cadherin controls erythropoiesis; a novel avenue to treat anemia.
Each second the bone marrow produces millions of red blood cells (RBCs). Aging-associated bone marrow diseases disturb this process leading to anemia thereby generating significant health issues. Chronic anemic patients receive blood transfusions that temporarily restore their RBC counts to a healthy threshold. Prolonged transfusion support during treatment of chronic anemia, increases however the risk of transfusion-related health complications. To prevent pathophysiological effects of chronic transfusion, patients with chronic anemia require additional treatment options to reduce transfusion support. These treatments would be based on the stimulation of the patients own production of RBCs that can be applied in combination with the support of blood cell transfusions. This research therefore aims to improve our understanding into the regulation of RBC production in bone marrow during anemia to develop novel treatment options to treat patients with chronic anemia.
2019 – Prof Arjan Griffioen / Dr Coert Margadant – Angiogenesis Laboratory Amsterdam – Amsterdam UMC – location VUmc / Sanquin
Effect of anti-HPA-1a antibodies on sprouting angiogenesis and bleeding in fetal and neonatal alloimmune thrombocytopenia.
Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a serious condition that can occur during pregnancy because components of the mother’s immune system, antibodies, attack the cells of the developing child. The consequences can be dramatic and include the occurrence of bleedings in the child’s brain, which may cause life-long disabilities or death around birth. Currently there is insufficient knowledge about the effects of these antibodies to explain why they cause bleeding and/or other complications in FNAIT. In the current project, we will investigate how the FNAIT antibodies function and why they cause complications. The results will be important to better understand FNAIT, and will make it possible to screen pregnant women in the future to identify those at high risk and treat them to prevent FNAIT.
2022 – Dr Sietse Nagelkerke – Blood Cell Research – Sanquin
Targeted ultralong-read sequencing to solve the genetic structure of complex genetic regions in blood group and immune genetics.
Genetic variation between individuals has a great influence on many illnesses. For instance, the chance to develop autoimmune diseases is influenced by genetic variation in genes of the immune system. In addition, genetic variation can influence the way different patients react to therapies, including blood transfusion and transfusion of human antibodies derived from blood. Knowledge of this genetic variation can help to develop new therapies and ensure patient-tailored treatment. However, some parts of the genome are very complex and the genetic variation is hard to investigate. This is especially true for genes of the immune system and some blood groups. We aim to develop a novel technique especially for our immune genes of interest, to determine genetic variation in great depth. This technique can also be employed to determine genetic variation underlying complex blood groups. The results of these studies can help to improve therapies and aid in matching of difficult blood groups.
2026 – Prof Jan Voorberg – Cellular Hemostasis – Sanquin
Conformational regulation of ADAMTS13 in immune trombotic thrombocytopenic purpura.
Immune mediated thrombotic thrombocytopenic purpura (TTP) provides a life-threatening disorder that is characterized by occlusion of small vessels by blood clots primarily in the brain and gastro-intestinal tract. Excessive formation of blood clots is due to the development of auto-antibodies that target ADAMTS13; ADAMTS13 controls primary hemostasis by limiting platelet plug formation at sites of vascular injury. The absence of ADAMTS13 may present with spontaneous formation of platelet-rich blood clots in the brain and gastro-intestinal tract which if left untreated can be fatal. At present we do not know why auto-antibodies directed towards ADAMTS13 develop in previously healthy individuals. The goal of the current project is to understand how pathogenic autoantibodies develop in these patients. This project may result in improved diagnostic tests which may predict relapses and may also result in novel treatment options for patients suffering from autoimmune TTP.
Decisions by the Boards of the LSBR (in Dutch) can be found on the page of our Annual Report (in Dutch).