A report detailing a severe case of hemolytic disease of the fetus and newborn (HDFN) that resulted in neonatal death 10 days after birth was recently published in the medical journal Transfusion.
The authors described a case of a patient with severe HDFN due to ABO incompatibility that led to death 10 days after birth despite the administration of appropriate treatment with transfusions and phototherapy.
HDFN is a consequence of the destruction of fetal red blood cells by maternal antibodies. These antibodies target specific proteins in the membrane of the red blood cells; these proteins are known as antigens. Not all antigens cause the same response; the Rh antigen, in particular, is known for producing a particularly strong immune response, leading to complications during and after pregnancy.
Since the introduction of preventive treatment with Rh immunoglobulin (RHIG) decades ago, the frequency of HDFN due to Rh incompatibility has fallen, making ABO antigen incompatibility the most common cause of the disease. Unlike Rh incompatibility, ABO rarely leads to severe disease.
Learn more about HDFN prognosis
The patient was born at full term after an uneventful pregnancy. The mother was O Rh negative, and the patient B positive; the mother had received appropriate prenatal care, including RHIG administration. However, the patient was born with anemia and high bilirubin levels (hyperbilirubinemia), both hallmarks of HDFN.
The patient was diagnosed with HDFN due to ABO incompatibility and was treated and discharged home as a healthy infant. Two days later, the patient presented diarrhea and vomiting and was referred to the emergency room. At the memento of admission, the patient had yellow coloration of the skin due to hyperbilirubinemia (jaundice) and anemia.
The patient developed apneic episodes and had to be put on respiratory support. Further testing revealed dead tissue in the bowel (necrosis), and it was extensively tested for infection. Despite transfusion, hemoglobin and platelet levels kept falling.
During the following days, the patient developed multiple organ failure and ultimately died on day 10 after birth.
The authors highlighted that the patient had four significant risk factors for developing severe HDFN: African-American ethnicity, group O mother and group B neonate, an unusually high expression of B antigen in the neonatal red blood cells and high titers of anti B antibodies.
“Although ABO HDN is usually a mild condition commonly encountered by pediatricians and transfusion medicine specialists, cases can include severe, atypical presentations that are associated with distinctive laboratory and clinical features.” the authors concluded.