Pregnant Patient With Sickle Cell Disease And Anti-Fy3

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 nonAfricans.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 patient ' s 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.