Hereditary spherocytosis

There is limited information about the effect of hereditary spherocytosis (HS) on pregnancy. Hemolytic crisis and persistent anemia have been reported during pregnancy, especially in women who have not undergone splenectomy.^[1]

One article reported on 8 patients with HS who had a total of 19 pregnancies:

• 10 pregnancies occurred in patients before splenectomy, and 9 occurred after splenectomy.
• There were 13 term births, 4 spontaneous abortions (miscarriages), and 2 therapeutic abortions (pregnancy termination for medical indications).
• Of the 19 pregnancies, 8 were complicated by anemia and all were in patients without splenectomy. A hemolytic crisis occurred in 6 pregnancies, and persistent anemia occurred in 2 pregnancies. Transfusion was required in 4 pregnancies.^[2]

It would appear that pregnancy may cause hemolytic anemia, but maternal morbidity and fetal outcome seem more favorable after splenectomy than before splenectomy.^[2]

In terms of pregnancy management, folic acid supplementation is necessary. Monitoring for worsening of anemia with complete blood counts and reticulocyte counts is recommended.^[1]

Few studies have investigated the effect of splenectomy on pregnancy. Most of the studies performed looked at neonatal outcome among women with immune thrombocytopenia (ITP) who have had a splenectomy, and very few have focused on obstetric outcome.

A study that investigated pregnancy in patients with hereditary spherocytosis (HS) found that only about one third of pregnancies in non-splenectomized women developed anemia, or anemia deteriorated. The authors noted that in splenectomized patients, the incidence of complaints was minimal.^[3]

There has been one retrospective study comparing pregnancies of women who have and have not undergone splenectomy (not specific to women with HS). The major finding of this study was that splenectomy is a significant risk factor for adverse obstetric outcomes, and specifically, is an independent risk factor for preterm delivery. However, while there were higher rates of preterm delivery, these deliveries were near term and had no impact on birth outcome.^[4]

Obstetric complications associated with splenectomy included C-section, maternal blood transfusion, pneumonia during pregnancy, and unspecified complications of anesthesia and sedation during labor. Higher rates of fertility treatments were also found among post-splenectomy women.^[4]

Importantly, pregnancies following splenectomy were not associated with adverse perinatal outcome - no significant differences were found between the groups regarding low Apgar scores, congenital malformations (birth defects), intrauterine growth restriction (IUGR), or perinatal death.^[4]