Article
Biochemistry & Molecular Biology
Thijs W. de Vos, Dian Winkelhorst, Hans J. Baelde, Kyra L. Dijkstra, Rianne D. M. van Bergen, Lotte E. van der Meeren, Peter G. J. Nikkels, Leendert Porcelijn, C. Ellen van der Schoot, Gestur Vidarsson, Michael Eikmans, Rick Kapur, Carin van der Keur, Leendert A. Trouw, Dick Oepkes, Enrico Lopriore, Marie-Louise P. van der Hoorn, Manon Bos, Masja de Haas
Summary: This study found that untreated FNAIT cases showed more C4d deposition in the placenta, which may impact placental function and fetal growth. Histopathological examination revealed a certain proportion of placental delayed maturation in these cases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Obstetrics & Gynecology
James B. Bussel, Emilie L. Vander Haar, Richard L. Berkowitz
Summary: Advances in managing fetal and neonatal alloimmune thrombocytopenia may include screening all antepartum patients, noninvasively testing fetal human platelet antigen 1 genotype, developing a prophylactic product equivalent to Rh immune globulin, and creating neonatal Fc receptor inhibitors as potential therapies.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2021)
Article
Hematology
Assaf Barg, Lilach Bonstein
Summary: This review discusses the advances in the field of fetal and neonatal alloimmune thrombocytopenia (FNAIT), including new methods for prenatal diagnosis and treatment. Topics explored include noninvasive prenatal testing, novel therapeutic options developed through the use of mouse models, and research on the biological characteristics of alloantibodies and their association with the risk of fetal bleeding.
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2023)
Article
Immunology
Zoltan Szittner, Arthur E. H. Bentlage, A. Robin Temming, David E. Schmidt, Remco Visser, Suzanne Lissenberg-Thunnissen, Juk Yee Mok, Wim J. E. van Esch, Myrthe E. Sonneveld, Erik L. de Graaf, Manfred Wuhrer, Leendert Porcelijn, Masja de Haas, C. Ellen van der Schoot, Gestur Vidarsson
Summary: The core fucosylation in anti-HPA-1a IgG greatly influences its binding to leukocyte IgG-Fc receptors IIIa/b. A cellular surface plasmon resonance imaging technique was developed to quantify the biological activity of IgG antibodies targeting cells.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Hematology
Thijs W. de Vos, Leendert Porcelijn, Suzanne Hofstede-van Egmond, Eva Pajkrt, Dick Oepkes, Enrico Lopriore, C. Ellen van der Schoot, Dian Winkelhorst, Masja de Haas
Summary: Fetal neonatal alloimmune thrombocytopenia (FNAIT) is a condition caused by maternal alloantibodies targeting human platelet antigens, which can result in severe bleeding in the unborn child. While anti-HPA-1a-mediated FNAIT tends to have a more severe clinical outcome, anti-HPA-5b may also be associated with severe neonatal bleeding symptoms. Further research, including a prospective study, is needed to fully understand the natural history of anti-HPA-5b mediated FNAIT.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Endocrinology & Metabolism
Juliane Leger, Clemence Delcour, Jean-Claude Carel
Summary: This article discusses the diagnosis and management of fetal and neonatal thyroid dysfunctions, including congenital hypothyroidism, autoimmune hyperthyroidism, and other rare disorders. Early diagnosis and treatment are crucial in preventing irreversible neurodevelopmental delay.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Review
Hematology
Julia Alm, Yalin Duong, Sandra Wienzek-Lischka, Nina Cooper, Sentot Santoso, Ulrich J. Sachs, Volker Kiefel, Gregor Bein
Summary: Most cases of FNAIT are caused by maternal anti-HPA-1a antibodies, while anti-HPA-5b antibodies are the second most common antibodies. However, there is no evidence supporting the idea that anti-HPA-5b antibodies cause severe thrombocytopenia or bleeding complications.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Cell Biology
Rebecca Carter, Anna-Kaisa Niemi
Summary: Noonan syndrome is a genetic disorder with distinctive physical features and often involves bleeding disorders. This case report presents a neonate with intracranial hemorrhage and severe thrombocytopenia, found to have both NAIT and a de novo heterozygous pathogenic variant in PTPN11, consistent with Noonan syndrome.
Review
Hematology
Wendy Stam, Gabriela Elis Wachholz, Jose Maria de Pereda, Rick Kapur, Ellen van der Schoot, Coert Margadant
Summary: FNAIT is a pregnancy-associated condition caused by maternal alloantibodies against paternally-inherited platelet antigens, most frequently HPA-1a on integrin beta 3. The clinical effects range from no symptoms to fatal intracranial hemorrhage, but underlying pathophysiological determinants are poorly understood.
Article
Hematology
David Boehm, Sandra Wienzek-Lischka, Nina Cooper, Heike Berghoefer, Katja Mueller, Behnaz Bayat, Gregor Bein, Ulrich J. Sachs
Summary: In fetal/neonatal alloimmune thrombocytopenia (FNAIT), maternal alloantibodies against paternal human platelet antigens (HPA) lead to platelet destruction through the placenta. The extent of thrombocytopenia varies, and inflammation may play a role. In this study, inflammatory markers were measured in serum samples from neonates with low platelet counts, and it was found that systemic inflammation did not contribute significantly to thrombocytopenia in FNAIT. However, the antiangiogenic enzyme sFlt-1 released by the placenta correlated with platelet count in FNAIT cases, suggesting that placental inflammation may modulate disease severity.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Obstetrics & Gynecology
Thijs W. de Vos, Masja de Haas, Dick Oepkes, Ratna N. G. B. Tan, C. Ellen van der Schoot, Sylke J. Steggerda, Linda S. de Vries, Enrico Lopriore, Jeanine M. M. van Klink
Summary: This study aimed to evaluate the long-term neurodevelopmental outcomes in children with fetal and neonatal alloimmune thrombocytopenia who were treated with antenatal intravenous immunoglobulin. The results showed that the risk of neurodevelopmental impairment in these children is comparable to that reported in the general population.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2022)
Article
Obstetrics & Gynecology
Siw L. Ernstsen, Maria T. Ahlen, Tiril Johansen, Eirin L. Bertelsen, Jens Kjeldsen-Kragh, Heidi Tiller
Summary: This study compared the outcomes of pregnancies with anti-human platelet antigen-1a-induced intracranial hemorrhage treated with intravenous immunoglobulin versus pregnancies without this treatment. The results showed that omitting antenatal intravenous immunoglobulin treatment in low-risk pregnancies does not increase the risk of neonatal intracranial hemorrhage.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Jorge Lopez-Tello, Zoe Schofield, Raymond Kiu, Matthew J. Dalby, Douwe van Sinderen, Gwenaelle Le Gall, Amanda N. Sferruzzi-Perri, Lindsay J. Hall
Summary: This study demonstrates that the bacterium Bifidobacterium breve UCC2003, a key member of the maternal gut microbiota, modulates maternal body adaptations, placental structure, and nutrient transporter capacity, with implications for fetal metabolism and growth.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2022)
Article
Hematology
Kristine Matusiak, Christopher J. Patriquin, Stacy Deniz, Nancy Dzaja, James W. Smith, Grace Wang, Ishac Nazy, John G. Kelton, Donald M. Arnold
Summary: This study investigated clinical and laboratory predictors of severe FNAIT and found that maternal antibodies to HPA were the only independent predictor of severe FNAIT in subsequent pregnancies. However, one infant had a severe FNAIT recurrence without maternal antibodies, highlighting the need for improved prevention and treatment strategies.
Review
Medicine, General & Internal
Shigeo Iijima
Summary: Cytomegalovirus (CMV) is the most common cause of intrauterine infection during pregnancy. Serological assays, including CMV-specific immunoglobulin M (IgM) antibodies, are used to assess CMV infections. IgG avidity testing can help differentiate between primary and non-primary infections, but there is no standardized assay for this. Maternal serology may show positive CMV-IgG with negative CMV-IgM results, leading to exclusion of vertical transmission probability, but recent cases have shown symptomatic congenital infections in the absence of CMV-IgM.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Hematology
David E. Schmidt, Anke J. Lakerveld, Katja M. J. Heitink-Polle, Marrie C. A. Bruin, Gestur Vidarsson, Leendert Porcelijn, Masja de Haas
Article
Multidisciplinary Sciences
David E. Schmidt, Noortje de Haan, Myrthe E. Sonneveld, Leendert Porcelijn, C. Ellen van der Schoot, Masja de Haas, Jaap-Jan Zwaginga, Manfred Wuhrer, Gestur Vidarsson
SCIENTIFIC REPORTS
(2020)
Article
Hematology
David E. Schmidt, Pernille Wendtland Edslev, Katja M. J. Heitink-Polle, Bart Mertens, Marrie C. A. Bruin, Rick Kapur, Gestur Vidarsson, C. Ellen van der Schoot, Leendert Porcelijn, Johanna G. van der Bom, Steen Rosthoj, Masja de Haas
Summary: The Childhood ITP Recovery Score was developed to predict transient versus persistent ITP in children with newly diagnosed ITP. The score showed good predictive performance in external validation and could be valuable for guiding clinical management.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
David E. Schmidt, Katja M. J. Heitink-Polle, Bart Mertens, Leendert Porcelijn, Rick Kapur, C. Ellen van Der Schoot, Gestur Vidarsson, Johanna G. van Der Bom, Marrie C. A. Bruin, Masja de Haas
Summary: This study focused on predicting the response to IVIg treatment and ITP disease course in children, identifying 5 variables that predict ASR vs CSR, which improved discrimination of favorable ITP disease courses.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
Thijs W. de Vos, Leendert Porcelijn, Suzanne Hofstede-van Egmond, Eva Pajkrt, Dick Oepkes, Enrico Lopriore, C. Ellen van der Schoot, Dian Winkelhorst, Masja de Haas
Summary: Fetal neonatal alloimmune thrombocytopenia (FNAIT) is a condition caused by maternal alloantibodies targeting human platelet antigens, which can result in severe bleeding in the unborn child. While anti-HPA-1a-mediated FNAIT tends to have a more severe clinical outcome, anti-HPA-5b may also be associated with severe neonatal bleeding symptoms. Further research, including a prospective study, is needed to fully understand the natural history of anti-HPA-5b mediated FNAIT.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Thijs W. de Vos, Dian Winkelhorst, Hans J. Baelde, Kyra L. Dijkstra, Rianne D. M. van Bergen, Lotte E. van der Meeren, Peter G. J. Nikkels, Leendert Porcelijn, C. Ellen van der Schoot, Gestur Vidarsson, Michael Eikmans, Rick Kapur, Carin van der Keur, Leendert A. Trouw, Dick Oepkes, Enrico Lopriore, Marie-Louise P. van der Hoorn, Manon Bos, Masja de Haas
Summary: This study found that untreated FNAIT cases showed more C4d deposition in the placenta, which may impact placental function and fetal growth. Histopathological examination revealed a certain proportion of placental delayed maturation in these cases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Medicine, General & Internal
Anne-Tess C. Jolink, Vivianne S. Nelson, Martin R. Schipperus, Sufia N. Amini, Gestur Vidarsson, C. Ellen van der Schoot, Leendert Porcelijn, Masja de Haas, Rick Kapur
Summary: Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by platelet destruction and MK impairment. The challenging therapeutic management and potential complications underscore the importance of research into personalized treatment strategies and predictive markers for treatment responses.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Cell Biology
Vivianne S. Nelson, Anne-Tess C. Jolink, Sufia N. Amini, Jaap Jan Zwaginga, Tanja Netelenbos, John W. Semple, Leendert Porcelijn, Masja de Haas, Martin R. Schipperus, Rick Kapur
Summary: ITP is an autoimmune bleeding disorder characterized by low platelet levels, involving mechanisms like autoantibody-dependent and cytotoxic T cell mediated platelet clearance. Interestingly, platelets not only mediate hemostasis, but also modulate inflammatory and immunological processes, potentially playing an immuno-modulatory role in ITP. Platelets and platelet microparticles may be involved in promoting inflammation, stimulating immune responses, and contributing to disrupted immune balance and impaired thrombopoiesis in ITP, providing new avenues for research and potential therapies.
Article
Hematology
Sufia N. Amini, Leendert Porcelijn, Annemieke Sobels, Marina Kartachova, Masja de Haas, Jaap Jan Zwaginga, Martin R. Schipperus
Summary: This study investigated the association between anti-GP antibodies and platelet sequestration patterns and clearance rate in ITP patients. The results showed that anti-GPV antibodies are associated with a splenic sequestration pattern, and in patients where antibodies were present, the clearance rate was higher in patients with a splenic sequestration.
Article
Hematology
Thijs L. J. van Osch, Janita J. Oosterhoff, Arthur E. H. Bentlage, Jan Nouta, Carolien A. M. Koeleman, Dionne M. Geerdes, Juk Yee Mok, Sebastiaan Heidt, Arend Mulder, Wim J. E. van Esch, Rick Kapur, Leendert Porcelijn, Ellen van der Schoot, Masja de Haas, Manfred Wuhrer, Jan Voorberg, Gestur Vidarsson
Summary: Approximately 20% of patients receiving multiple platelet transfusions develop platelet alloantibodies, which can lead to platelet refractoriness. Factors such as antibody glycosylation and the breadth of the polyclonal immune response may affect complement activation and clearance of donor platelets.
Letter
Hematology
T. Langerak, G. J. Bakker, L. Porcelijn, M. N. Lauw, R. J. van de Laar, M. Eefting
THROMBOSIS RESEARCH
(2022)
Article
Pediatrics
Inez M. Vanholder, Ginette M. Ecury-Goossen, Jop Admiraal, Leendert Porcelijn, Rosalina Ml van Spaendonk, Samantha C. Gouw
JOURNAL OF PAEDIATRICS AND CHILD HEALTH
(2022)
Editorial Material
Hematology
Sufia N. Amini, Vivianne S. Nelson, Leendert Porcelijn, Tanja Netelenbos, Jaap Jan Zwaginga, Masja de Haas, Martin R. Schipperus, Rick Kapur
Summary: ITP is an autoimmune bleeding disorder characterized by platelet clearance in the spleen and liver. Thrombopoietin (TPO) levels remain low in ITP. The role of GPIb antibodies in relation to platelet hepatic sequestration and TPO levels in ITP remains unclear. This study found a positive association between GPIb antibodies and TPO levels as well as platelet hepatic sequestration in patients with severe thrombocytopenic ITP.
Article
Hematology
Thijs L. J. van Osch, Tamas Pongracz, Dionne M. Geerdes, Juk Yee Mok, Wim J. E. van Esch, Jan Voorberg, Rick Kapur, Leendert Porcelijn, Jean-Louis H. Kerkhoffs, Pieter F. van Der Meer, C. Ellen van Der Schoot, Masja de Haas, Manfred Wuhrer, Gestur Vidarsson
Summary: The Fc glycosylation profile of anti-HLA class I antibodies formed after platelet transfusion varies between patients, and could potentially explain the variation in clinical severity.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Thijs W. de Vos, Dian Winkelhorst, Valgerdur Arnadottir, Johanna G. van Der Bom, Carme Canals Suris, Camila Caram-Deelder, Emoke Deschmann, Helen E. Haysom, Hem Birgit C. Hverven, Jana Lozar Krivec, Zoe K. McQuilten, Eduardo Muniz-Diaz, Nuria Nogues, Dick Oepkes, Leendert Porcelijn, C. Ellen van Der Schoot, Matthew Saxonhouse, Martha Sola-Visner, Eleonor Tiblad, Heidi Tiller, Erica M. Wood, Vanessa Young, Mojca Zeleznik, Masja de Haas, Enrico Lopriore
Summary: Management of FNAIT varies greatly between international centres, with HPA-matched transfusions resulting in a larger median platelet count increment than HPA-unmatched transfusions, but whether HPA matching is also associated with a reduced risk of bleeding remains unknown.
LANCET HAEMATOLOGY
(2022)