title: Fetal/Neonatal Alloimmune Thrombocytopenia: Pathogenesis, Diagnostics and Prevention.
abstract: Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a relatively rare condition (1/1000-1/2000) that was granted orphan status by the European Medicines Agency in 2011. Clinical consequences of FNAIT, however, may be severe. A thrombocytopenic fetus or new-born is at risk of intracranial hemorrhage that may result in lifelong disability or death. Preventing such bleeding is thus vital and requires a solution. Anti-HPA1a antibodies are the most frequent cause of FNAIT in Caucasians. Its pathogenesis is similar to hemolytic disease of the newborn (HDN) due to anti-RhD antibodies, but is characterized by platelet destruction and is more often observed in the first pregnancy. In 75 % of these women, alloimmunization by HPA-1a antigens, however, occurs at delivery, which enables development of antibody-mediated immune suppression to prevent maternal immunization. As for HDN, the recurrence rate of FNAIT is high. For advancing diagnostic efforts and treatment, it is thereby crucial to understand the pathogenesis of FNAIT, including cellular immunity involvement. This review presents the current knowledge on FNAIT. Also described is a program for HPA-1a screening in identifying HPA-1a negative pregnant women at risk of immunization. This program is now performed at the Institute of Hematology and Transfusion Medicine in cooperation with the Department of Obstetrics and Gynecology of the Medical Centre of Postgraduate Education in Warsaw as well as the UiT The Arctic University of Norway.
authors.affiliation: Department of Immunohematology and Immunology of Transfusion Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland. ebrojer@ihit.waw.pl.
authors.forename: Ewa
authors.identifier: http://orcid.org/0000-0003-1947-6888
authors.initials: E
authors.lastname: Brojer
authors.affiliation: Institute of Medical Biology, UiT The Arctic University of Norway, Tromsø, Norway.
authors.forename: Anne
authors.initials: A
authors.lastname: Husebekk
authors.affiliation: 2nd Department of Obstetrics and Gynecology, Medical Centre of Postgraduate Education, Warsaw, Poland.
authors.forename: Marzena
authors.initials: M
authors.lastname: Dębska
authors.affiliation: Department of Immunohematology and Immunology of Transfusion Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Uhrynowska
authors.affiliation: Department of Immunohematology and Immunology of Transfusion Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Guz
authors.affiliation: Department of Immunohematology and Immunology of Transfusion Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Orzińska
authors.affiliation: 2nd Department of Obstetrics and Gynecology, Medical Centre of Postgraduate Education, Warsaw, Poland.
authors.forename: Romuald
authors.initials: R
authors.lastname: Dębski
authors.affiliation: Department of Immunohematology and Immunology of Transfusion Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.
authors.forename: Krystyna
authors.initials: K
authors.lastname: Maślanka
mesh_terms: D017951:Antigen Presentation; D016824:Antigens, Human Platelet / Q000276:immunology; D001792:Blood Platelets / Q000166:cytology; D005060:Europe; D005260:Female; D006470:Hemorrhage / Q000503:physiopathology; D006801:Humans; D007111:Immunity, Cellular; D056724:Immunity, Humoral; D007231:Infant, Newborn; D039661:Integrin beta3; D007519:Isoantigens / Q000276:immunology; D008297:Male; D015997:Neonatal Screening / Q000379:methods; D011044:Poland; D011247:Pregnancy; D012204:Rh-Hr Blood-Group System; D054098:Thrombocytopenia, Neonatal Alloimmune / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology* / Q000517:prevention & control*
chemical_list: D016824:Antigens, Human Platelet; C061248:ITGB3 protein, human; D039661:Integrin beta3; D007519:Isoantigens; D012204:Rh-Hr Blood-Group System
keywords: Fetal/neonatal alloimmune thrombocytopenia; Human platelet alloantigens; Pregnancy; Therapeutic intervention