Severe refractory ß-thalassemia and hypersplenism in pregnancy: a case report

ß-thalassemia intermedia in pregnant patients can present as severe anemia and increased risk for fetal growth restriction requiring frequent transfusions. However, there is little data on the presentation of ß-thalassemia intermedia in pregnancy refractory to repeated transfusions and no clinical guidance on antepartum management of this severe presentation. This case report describes the clinical presentation and management of a patient with ß-thalassemia intermedia who presented with anemia refractory to transfusions and severe hypersplenism in her second trimester of pregnancy. A 36-year-old woman, gravida 2 para 1, at 17 3/7 weeks of gestation with ß-thalassemia intermedia and transfusion dependence during her prior pregnancy presented with hemoglobin 4.9 g/dL despite recent transfusion and significant splenomegaly confirmed by ultrasonography. The patient received multiple packed red blood cell transfusions with modest improvement but subsequently failed further crossmatches due to development of an alloantibody. Following interdisciplinary team meetings with the patient, open splenectomy was performed at 19 3/7 weeks of gestation without complications. Pathology was consistent with hypersplenism due to extramedullary hematopoiesis. Post-operatively, the patient responded well to transfusions and delivered a liveborn female at 37 2/7 weeks of gestation via induced vaginal delivery. This case report describes severe ß-thalassemia intermedia in pregnancy complicated by hypersplenism, refractory to repeated transfusions. Splenectomy in the second trimester can be a safe management option for severe transfusion-refractory anemia in pregnant patients with ß-thalassemia. Given the complexity of patient care and involved risks, careful shared decision-making with an interdisciplinary team is strongly recommended.