Fetale und neonatale Alloimmunthrombozytopenie, Teil 1

Zusammenfassung

Die fetale und neonatale Alloimmunthrombozytopenie (FNAIT) wird durch mütterliche Antikörper hervorgerufen, die gegen ein vom Vater ererbtes Blutgruppenmerkmal an fetalen Thrombozyten gerichtet sind. Teil 1 des Beitrags stellt die Ätiologie, die Pathogenese und die Diagnostik der FNAIT dar, während Teil 2 die Risikostratifizierung und Behandlung der FNAIT thematisiert [1].

Abstract

Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a bleeding condition caused by maternal antibodies against fetal platelet antigens inherited from the father. The incidence of severe FNAIT (neonatal platelet count < 50 G/l) is 1 in 2500 live births. Its most severe consequence, intracranial hemorrhage, leading to death or neurologic disability, occurs in 10 per 100 000 neonates, commonly prior to birth. This review summarizes the current knowledge on incidence, pathogenesis, diagnosis of FNAIT in this part 1 followed by the management of pregnancies at risk and prophylaxis in part 2.

Kernaussagen

• Die fetale und neonatale Alloimmunthrombozytopenie (FNAIT) wird durch mütterliche Antikörper hervorgerufen, die gegen ein vom Vater ererbtes Blutgruppenmerkmal an fetalen Thrombozyten gerichtet sind.

• Die FNAIT wird in ca. 80% der Fälle durch mütterliche Anti-HPA-1a-Antikörper ausgelöst, gefolgt von Anti-HPA-5b Antikörpern (ca. 15%). Rund zwei Drittel der Schwangeren werden in der 1. Schwangerschaft immunisiert. Die Inzidenz der Immunisierung gegen HPA-1a wird mit 1 : 500 angegeben.

• Gefürchtet ist eine fetale intrakranielle Blutung, die eine Inzidenz von 1 : 10 000 hat. Intrakranielle Blutungen treten meist früh in der Schwangerschaft auf, in über 50% der Fälle vor der 28. Schwangerschaftswoche. Peripartale Blutungen sind selten.

• Bei Neugeborenen mit isolierter Thrombozytopenie ist eine FNAIT die wichtigste Differenzialdiagnose.

• Bei klinischem Verdacht auf eine FNAIT und negativem HPA-Antikörpersuchtest muss ein Cross-Match zwischen mütterlichem Serum und paternalen Thrombozyten durchgeführt werden.

Schlüsselwörter

fetale und neonatale Alloimmunthrombozytopenie - FNAIT - neonatale Thrombozytopenie - nichtinvasive Pränataldiagnostik

Key words

fetal and neonatal alloimmune thrombocytopenia - FNAIT - neonatal thrombocytopenia - non-invasive prenatal diagnosis

Publikationsverlauf

Artikel online veröffentlicht:
01. Juni 2021

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