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Transfusionsmedizin 2013; 3(2): 79-81
DOI: 10.1055/s-0032-1324991
Kasuistik
Georg Thieme Verlag KG Stuttgart · New York

Schwangere Patientin mit Sichelzellenanämie und Anti-Fy3

Pregnant Patient with Sickle Cell Disease and Anti-Fy3
S. Flommersfeld
Zentrum für Transfusionsmedizin und Hämotherapie, Universitätsklinikum Gießen und Marburg, Standort Marburg
,
A. Korb-Bangang
Zentrum für Transfusionsmedizin und Hämotherapie, Universitätsklinikum Gießen und Marburg, Standort Marburg
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Publikationsverlauf

Publikationsdatum:
16. Mai 2013 (online)

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Zusammenfassung

Wir berichten über den Verlauf einer 39-jährigen Patientin mit bekannter homozygoter Sichelzellenanämie in ihrer 2. Schwangerschaft mit Anti-Fy3 und diversen anderen irregulären erythrozytären Antikörpern.

Abstract

Anti-Fy3 is a rare antibody mostly present in African patients of the Fy(a−b−) phenotype who were exposed to Fy-positive RBCs. Supply with RBC is difficult in Europe due to the fact that the Fy(a−b−) phenotype is extremely rare in non-Africans.

A 39-year-old female patient with homozygous sickle cell disease (and an average of 3–4 crises per year) presented in her 2nd pregnancy at the 7th week of gestation (WOG) with an initial Hb of 6.9 g/dl and reticulocyte counts of 83 ‰. She was known to be immunized against Fy3, Jkb, M, Leb, E and Doa from previous presentations. The father of her second child was of Caucasian extraction. At the initial presentation, Anti-M was the only detectable antibody. Further antibody screen plus ultrasounds (peak systolic flow velocity of the middle cerebral artery; PSFV) were performed every 10 days. Antibiotics were administered orally to avoid infection-triggered sickle cell crisis. In the 20th WOG an acute crisis led to a drop in Hb to 3.5 g/dl. Transfusion with one unit of selected, cross-match negative RBCs raised the patientʼs Hb to 5.9 g/dl. Four weeks later, a second crisis occurred (Hb 4.5 g/dl), and the following week, the patient was readmitted to hospital with stillbirth, (Hb of 4.8 g/dl). The Hb dropped to 2.8 g/dl post partum. The patient remained untransfused because she lacked clinical signs of anemia. Hb rose to 6 g/dl after 3 weeks. Pathological examination of the fetus and the placenta revealed placental insufficiency with spontaneous thrombosis.

Schlüsselwörter

Sichelzellanämie - Morbus hämolyticus neonatorum - seltene Blutgruppen

Key words

sickle cell disease - hemolytic disease of the newborn - rare blood groups