Ruptured Spleen in Newborn with Hemophilia A Treated Without Surgery

Margarida Azevedo, MSc avatar

by Margarida Azevedo, MSc |

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newborns and hemophilia

Ruptured spleens are rare in newborns, usually occurring secondary to an underlying coagulation disorder, such as hemophilia, or a splenic abnormality. Here, researchers report the first case of a newborn with a spontaneous splenic rupture and severe hemophilia A who was successfully treated without surgical intervention.

The case study, “Successful medical management of a neonate with spontaneous splenic rupture and severe hemophilia A,” was published in Hematology/Oncology and Stem Cell Therapy.

Splenic ruptures can be managed through a series of measures, from conservative treatment to surgery (splenectomy). In newborns with severe hemophilia, the most common coagulation disorder found in infants, an essential treatment is the replacement of factor concentrates ( factor VIII and factor IX, deficient in people with hemophilia A and B, respectively) and maintenance of these factors’ levels.

Researchers at the Ann & Robert H. Lurie Children’s Hospital of Chicago and Northwestern University Feinberg School of Medicine report the case of a newborn that, at day 4, was brought to the hospital’s emergency room (ER) and diagnosed with hemolytic disease of the newborn (HDN) secondary to ABO incompatibility, a condition in which the maternal antibodies pass through the placenta and into fetal circulation, destroying the fetus’ own red blood cells. Following treatment with a packed red blood cell transfusion (15 mL/kg), one dose of intravenous immunoglobulins (500 mg/kg/dose), and  two days of phototherapy, the baby was discharged.

Six days later, the infant returned to the ER and coagulation screening tests detected very low coagulation factor VIII activity, pointing to a hemophilia A diagnosis. Hematoma and bleeding from the spleen were observed through abdominal ultrasound and computerized tomography. The baby was treated with  recombinant factor VIII (rFVIII) concentrate at 50 IU/kg/dose every 8 hours (up to 72 hours) and given a second red blood cell transfusion, followed by changes in the treatment as symptoms improved.

“Based on our literature review, we have reported on the case of the first neonate with spontaneous splenic rupture and severe hemophilia A who underwent successful medical treatment without surgical intervention. In newborns with hemophilia or other bleeding disorders, appropriate replacement of exogenous factor concentrates could prevent emergency splenectomy,” the researchers concluded.