%0 Case Reports
%T Fatal hemolytic disease of the newborn due to anti-B Isohemagglutinin: An unfamiliar presentation of a familiar disease.
%A Longacre M
%A Bendapudi PK
%J Transfusion
%V 64
%N 1
%D 2024 01 8
%M 38069508
%F 3.337
%R 10.1111/trf.17614
%X Hemolytic disease of the newborn (HDN) occurs in approximately 1 out of 3000 live births. Severe presentations are atypical but must be recognized and treated rapidly to avoid life-threatening organ dysfunction.<BR>Here we report an unusual case of neonatal ABO HDN that illustrates the enormous inflammatory potential of maternal-fetal blood group mismatch. Following an uneventful delivery notable only for HDN caused by maternal anti-B IgG, our patient developed shock, DIC, and renal failure. Despite numerous interventions, she experienced a rapid clinical decline and died 10 days after birth. Treatment with whole blood exchange and a monoclonal antibody directed at complement component 5 (eculizumab) were attempted late in the disease course but were unsuccessful. Importantly, this patient had several known risk factors for severe ABO HDN, including the pentad of a group O mother with a group B neonate, high newborn red blood cell B antigen expression, presence maternal anti-B isohemagglutinin in high titer, presence of a maternal IgG anti-B isohemagglutinin, and African ancestry.<BR>Clinicians should be aware of the potential for severe ABO HDN and consider earlier diagnostic workup and more aggressive therapy in patients with high-risk features.