Frühe und schwer verlaufende hämolytische Erkrankung des Fetus und Neugeborenen

Zusammenfassung

Eine schwere Alloimmunisierung der Schwangeren gegen erythrozytäre Blutgruppenmerkmale kann insbesondere bei Antikörpern der Spezifität Anti-K bereits vor der 20. Schwangerschaftswoche zu einer fetalen Anämie führen. Wir berichten über eine 37-jährige Schwangere (Gravida 6, Para 5) mit bekanntem Anti-K. In der Vorgeschichte kam es aufgrund einer K-Inkompatibilität in der 4. Schwangerschaft in der 34. Schwangerschaftswoche (SSW) zu einem Hydrops fetalis und in der 5. Schwangerschaft in der 22. SSW zu einem intrauterinen Fruchttod. Mittels eines nicht invasiven Pränataltests (NIPT) wurde in der aktuellen Schwangerschaft erneut ein K-positiver Fetus bestätigt. Aufgrund der Vorgeschichte erfolgte eine prophylaktische hochdosierte i. v. Immunglobulingabe (IVIG) (1 g/kgKG/Woche) ab der 14. SSW. In der 29 + 1 SSW war bei beginnender fetaler Anämie eine intrauterine Transfusion (IUT) erforderlich. Der weitere Schwangerschaftsverlauf gestaltete sich unter Fortführung der wöchentlichen IVIG-Therapie unauffällig. Das Kind kam in der 40 + 2 SSW durch Spontangeburt zur Welt. Bei positivem direktem Coombs-Test ließ sich im Eluat das mütterliche Anti-K nachweisen. Postpartal kam es bei dem Neugeborenen nur zu einem leichten Abfall der Hämoglobinkonzentration von 11,7 auf 9,5 g/dl. Eine Therapie war nicht erforderlich.

Abstract

Severe alloimmunization of pregnant women against antigens on red blood cells (RBC) can lead to fetal anemia even before the 20th week of gestation, especially if anti-K antibodies are present. We report of a 37-year-old pregnant woman (G₆P₅) with known anti-K. She had a past history of fetal hydrops at 34th week of gestation in the 4th pregnancy and an intrauterine fetal demise at the 22nd gestational week in her 5th pregnancy caused by K-incompatibility, respectively. Using non-invasive prenatal testing (NIPT), a K-positive fetus was confirmed in the current pregnancy. In view of the previous history, prophylactic high-dose i. v. immunoglobulin (IVIG) (1 g/kg bw/week) was administered from the 14th week of gestation onwards. Due to fetal anemia, an intrauterine transfusion (IUT) was necessary at gestational week 29 + 1. The further course of the pregnancy was inconspicuous, while continuing the weekly IVIG therapy. The child was delivered at 40 + 2 weeks of gestation by vaginal delivery. The direct Coombs test was positive, and maternal anti-K could be eluted from the newbornsʼ RBC. Post partum there was only a slight decrease in hemoglobin concentration from 11.7 g/dl to 9.5 g/dl with no therapy necessary.

Schlüsselwörter

hochdosierte intravenöse Immunglobulingabe - therapeutische Plasmapherese - erythrozytäre Alloimmunisierung in der Schwangerschaft - intrauteriner Kindstod

Key words

high-dose intravenous immunoglobulins - therapeutic plasmapheresis - RBC alloimmunization in pregnancy - fetal loss

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