A 22-year-old woman is seen for a routine prenatal evaluation; she is 10 weeks pregnant. This is her second pregnancy; the first pregnancy was uncomplicated. Medical history is notable for sickle cell disease requiring one to two hospitalizations per year for painful events. She has no history of stroke or acute chest syndrome. Her only medication is a folic acid supplement.
On physical examination, vital signs are normal. Cardiac examination reveals a grade 2/6 systolic flow murmur. She has a gravid uterus.
Laboratory studies show a hemoglobin level of 6.4 g/dL (64 g/L; baseline, 5-7 g/dL [50-70 g/L]), leukocyte count of 11,500/µL (11.5 × 109/L), and platelet count of 279,000/µL (279 × 109/L).
Which of the following is the most appropriate treatment?
B. Exchange transfusion throughout pregnancy
D. Simple transfusion throughout pregnancy
E. Expectant care
MKSAP Answer and Critique
The correct answer is E. Expectant care. This content is available to MKSAP 18 subscribers as Question 42 in the Hematology and Oncology section. More information about MKSAP is available online.
This patient requires no treatment other than expectant care. She should be closely monitored and treatment should be withheld until symptoms appear or some measurable parameter changes. Sickle cell disease (SCD) is associated with maternal morbidity and mortality from stroke, vaso-occlusive crisis, and acute chest syndrome. Furthermore, the risk of eclampsia is higher in pregnant women with SCD. Fetal complications are also more common in patients with SCD, including low birth weight, spontaneous abortion, early delivery, and growth retardation. No routine interventions have been proven to decrease these complications, so close follow-up by hematologic and obstetric specialists and prompt management of evolving problems is important. Pregnancy outcomes are difficult to predict in pregnant patients with SCD, but one of the best indicators is the outcome from a previous pregnancy, which is encouraging in this patient.
Antiplatelet drugs, such as clopidogrel, have been associated with increased risk of bleeding and ablatio placenta without any clear benefit in pregnant patients with SCD.
Although the role of transfusion in the management of pregnant patients with SCD lacks consensus, no clear benefit has been seen with either simple or exchange transfusion in patients with uncomplicated disease or pregnancy. However, pregnant patients with SCD who have worsening symptomatic anemia should undergo transfusion. Those with past or new severe obstetric or fetal complications, those with twins, or those with chronic organ dysfunction may also benefit from transfusion. The decision to transfuse must be balanced against the risks of transfusion, including alloimmunization and delayed transfusion reaction, as well as iron and volume overload. Because this patient has an uncomplicated pregnancy, exchange or simple transfusion is not indicated.
Hydroxyurea therapy results in decreased mortality in SCD and is indicated in patients with recurrent painful episodes, acute chest syndrome, and symptomatic anemia. However, because of its potential teratogenicity, hydroxyurea should not be administered during pregnancy.
Proper contraception before hydroxyurea initiation should be discussed with the nonpregnant patient. This patient has no indication for hydroxyurea treatment.
- Patients with sickle cell disease and uncomplicated pregnancy should be closely monitored and treatment withheld until symptoms appear or some measurable parameter changes.