Fetal-Onset Autoimmune Hemolytic Anemia and Thrombocytopenia Followed by Giant Cell Hepatitis: A Case Report.

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 5 weeks after birth. At 6 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.