Introduction While neonates may suffer from alloimmune or secondary autoimmune diseases caused by placental transfer of maternal antibodies, primary neonatal autoimmune disease is extremely rare. Case Presentation We present a newborn girl with fetal-onset autoimmune disease, featuring prenatal hydrothorax, progressive Coombs-positive autoimmune hemolytic anemia, and autoimmune thrombocytopenia secondary to autoantibodies against glycoprotein IIb/IIIa, which were not present in the mother. Red blood cells and platelets were repeatedly transfused during the first five weeks after birth. At six months of life, the infant displayed elevated liver enzymes and vitamin K deficiency coagulopathy due to giant cell hepatitis. There was a partial response to systemic steroids, intravenous immunoglobulins, and anti-CD20 antibodies. Complete genome analysis failed to detect a genetic cause. Conclusion This rare case indicates that infantile Evans syndrome can already evolve during fetal development.
Mußotter et al. (Tue,) studied this question.