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
