Abstract

Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a disease in pregnancy characterized by maternal alloantibodies directed against the human platelet antigen (HPA). These antibodies can cause intracranial hemorrhage (ICH) or other major bleeding resulting in lifelong handicaps or death. Optimal fetal care can be provided by timely identification of pregnancies at risk. However, this can only be done by routinely antenatal screening. Whether nationwide screening is cost-effective is still being debated. HPA-1a alloantibodies are estimated to be found in 1 in 400 pregnancies resulting in severe burden and fetal ICH in 1 in 10.000 pregnancies. Antenatal treatment is focused on the prevention of fetal ICH and consists of weekly maternal IVIg administration. In high-risk FNAIT treatment should be initiated at 12–18 weeks gestational age using high dosage and in standard-risk FNAIT at 20–28 weeks gestational age using a lower dosage. Postnatal prophylactic platelet transfusions are often given in case of severe thrombocytopenia to prevent bleedings. The optimal threshold and product for postnatal transfusion is not known and international consensus is lacking. In this review practical guidelines for antenatal and postnatal management are offered to clinicians that face the challenge of reducing the risk of bleeding in fetuses and infants affected by FNAIT.

Highlights

  • Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a rare but severe disease in pregnancy

  • This study showed that the outcome in children with FNAIT and exposed to maternal intravenous immunoglobulins (IVIg) treatment was similar to the normal population [68]

  • In high-risk FNAIT, antenatal IVIg treatment should be initiated at 12–18 weeks gestational age, using a high dosage

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Summary

Introduction

Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a rare but severe disease in pregnancy. FNAIT develops in pregnancy due to maternal alloimmunization and results in thrombocytopenia and a risk of bleeding in the fetus and neonate. FNAIT is the most frequent cause of thrombocytopenia in otherwise healthy born neonates with an incidence of approximately 1 in 1500 pregnancies [3]. As FNAIT is a rare condition and often not recognized by clinicians, this entails difficulties to determine the burden of this disease on population level [4]. In this review we aim to summarize the current knowledge about the epidemiology of FNAIT and provide an overview on antenatal and postnatal management strategies based on the most recent literature

Pathogenesis
Human platelet antigens
Immunization
Functional effects of antibodies
Epidemiology
Incidence of HPA-alloantibodies
Incidence of severe thrombocytopenia
Antenatal management
Risk stratification
Intrauterine transfusions
Corticosteroids
Mode and timing of delivery
Future treatment options
Transfusion threshold
Platelet product
Long-term outcome
Future perspectives
Towards routine HPA-screening in pregnancy to prevent FNAIT – the HIP study
Findings
Conclusion
Full Text
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