Dr Lucy Birtwistle4, Ms Anna Hicks3,4, Dr Kathryn Austin1, Dr Amanda Lazzaro1, Dr Natalie Cromer1,2
1Department of Obstetrics, Royal North Shore Hospital, St Leonards, Australia, 2Department of Haematology, Royal North Shore Hospital, St Leonards, Australia, 3University of Sydney, Camperdown, Australia, 4Royal North Shore Hospital, St Leonards, Australia
Biography:
Dr Lucy Birtwistle, a Junior Medical Officer at Royal North Shore Hospital, has a fervent passion for haematology and women's health.
Anna-Katarina Hicks, a second-year medical student at Sydney University, has pivoted her vocation from Jazz music to O&G.
Together they endeavour to improve clinical outcomes via translational research.
Background: Evans syndrome describes immune thrombocytopenia and warm autoimmune haemolytic anaemia (AIHA), with a positive direct antiglobulin test (DAT) and/or neutropenia.¹ IgG antibodies can cross the placenta, leading to neonatal thrombocytopenia and/or haemolysis.
Case Report: A 29-year-old nulliparous female presented at 20 weeks gestation with platelets of 47×10⁹/L, compensated autoimmune haemolysis, positive autoimmune markers, and was diagnosed with Evans syndrome. Treatment included hydroxychloroquine and one week of prednisone (0.5 mg/kg) to facilitate safe delivery at 37 weeks. The neonate had normal platelet (210×10⁹/L) and low-normal haemoglobin (140 g/L) levels, but had developed a cephalohaematoma. Despite sharing his mother’s blood group (B+), DAT was positive and jaundice progressed over four days, necessitating 48 hours of phototherapy. The mother had a flare of Evans syndrome at 18 months postpartum (nadir platelet 28×10⁹/L, haemoglobin 101 g/L with active haemolysis). Bone marrow biopsy excluded a lymphoproliferative or secondary cause. She responded to prednisone (1 mg/kg) with recurrence on weaning, so azathioprine was added to maintain partial remission and steroids weaned to minimise mood disturbance. Despite stable maternal platelet (>110×10⁹/L) and haemoglobin (>100 g/L) levels in her second pregnancy, fetal middle cerebral artery peak systolic flow (MCA-PSV) measured at the upper limit of normal for eight weeks, indicating fetal anaemia. Intravenous immunoglobulin (IVIg) treatment was prepared, but not initiated as MCA-PSV normalised. Born at 38+3 weeks, the neonate had normal platelet (178×10⁹/L) and haemoglobin (158 g/L) levels with spherocytosis, B+ blood group and DAT positivity. The infant developed jaundice within 22 hours with serum bilirubin (291 μmol/L) just below the exchange line. She required one week of quadruple-light phototherapy in the neonatal intensive care unit.
Conclusion: This case demonstrates transfer of autoantibodies of Evans syndrome can cause neonatal AIHA despite well-controlled maternal disease. Close monitoring and potential IVIg therapy are recommended to mitigate this risk.
Keywords
Evans syndrome, autoimmune haemolytic anaemia, immune thrombocytopenia, pregnancy, neonate
References
1. Eleftheria Lefkou, Nelson-Piercy C, Hunt BJ. Evans’ syndrome in pregnancy: A systematic literature review and two new cases. European journal of obstetrics, gynecology, and reproductive biology/European journal of obstetrics & gynecology and reproductive biology [Internet]. 2010 Mar 1 [cited 2024 May 27];149(1):10–7. Available from: https://www.ejog.org/article/S0301-2115(09)00700-3/abstract