Hematological complications of Gaucher's disease in pregnancy

Abstract

A case is presented of a 31-year-old Filipino female, gravida 5 para 2, at 38 weeks plus 5 days gestation, with known type I Gaucher's disease who underwent repeat cesarean delivery. After cesarean delivery, the patient developed disseminated intravascular coagulation and required transfusion of eight 6-packs of platelets, 6 units of fresh frozen plasma, two 10-packs of cryoprecipitate, and 6 units of packed red blood cells. Pregnancy is generally well tolerated in patients with type I Gaucher's disease, an autosomal recessive lysosomal storage disorder in which lipid deposits accumulate in the liver, spleen, and bone marrow. Hemorrhagic problems secondary to severe thrombocytopenia may develop postpartum in pregnancies complicated by Gaucher's disease, requiring significant support with blood and blood products.