Case Report: Evans Syndrome Coexisting with Sickle Cell Anaemia
Baba Jibrin¹*, Fatima I. Abubakar¹, Nma M. Jiya¹, Murtala M Ahmad¹, Muhammad A. Ndakotsu², Abubakar U. Musa², Fati B. Jiya¹, Asma'u Adamu¹, Khadija O. Isezuo¹
Introduction: Evans syndrome is a chronic disorder of immune regulation whose aetiology is unclear. It is characterized by a combination of autoantibodies directed against red blood cells, platelets and/or neutrophils, with typical presentation of chronic and relapsing autoimmune haemolytic anaemia, immune thrombocytopaenia with few cases of neutropenia. It affects all age groups and gender, response to therapy varies' but is often poor. Coexistence with comorbidities like SCA presents with difficulty in diagnosis due to their similarities in clinical features, hence diagnosis may be delayed, sometimes complicating each other's clinical presentation and often making management more challenging. Case Report: 11-year-old female, F.A known SCA patient who presented with fever, cough, jaundice, passage of dark brown coloured urine, epistaxis, petechia rashes and clinical features of anaemic heart failure. She had 6 blood transfusions and one exchange blood transfusion within 4 weeks of admission with worsening of symptoms. Subsequently developed right sided weakness and blindness. Serial FBC and, peripheral blood film showed severe cytopaenia (anaemia and thrombocytopenia), spherocytosis and, reticulocytosis. Direct coomb's test was positive while indirect coomb's test was negative. Bone marrow examination showed spherocytosis, LFT showed normal levels of enzymes with increased total and direct bilirubin. Brain CT revealed left occipital lobe infarcts with moderate cerebral atrophy. Prednisolone was commenced, patient responded well with improvement in the general health condition, resolution of motor weakness and cortical blindness. Follow up serial FBC showed increasing haematocrit, neutrophils, but with mild persistent thrombocytopenia which remained asymptomatic. Conclusion: Evans syndrome is a rare disease with a diagnostic challenge and variable response to treatment. High index of suspicion should be the key in SCA