Autoimmune Haemolytic Anaemia as Initial and Only Presentation of Systemic Lupus Erythematosus: A Case Report

Abstract

Autoimmune haemolytic anaemia (AIHA) is an acquired disorder resulting in the presence of antibodies against red blood cell antigens which leads to intravascular haemolysis. It can occur as the initial and sometimes the only presenting feature of Systemic Lupus Erythematosus (SLE). In this report, we describe the diagnostic evaluation and ongoing management of a 13-year-old female with AIHA as the first clinical feature of SLE. She presented with paleness of the body and yellowish discoloration of the eyes. Initial examination showed severe pallor with moderate jaundice. Investigations revealed severe anaemia with slightly elevated reticulocyte count and  positive direct coombs test(DCT).  Peripheral blood film showed microcytic, hypochromic anaemia. Bone marrow aspiration showed a hypocellular marrow for the age of the child. Immunological screening results were positive for antinuclear antibody (ANA) and anti-double stranded DNA (Anti-ds DNA). Patient is currently on Prednisolone, hydroxychloroquine and mycophenolate mofetil(MMF). The diagnosis of SLE with AIHA requires a good history, physical examination, high index of suspicion and extensive investigations. Management entails multidisciplinary approach, supportive care, initiation of treatment with steroids and  immunosuppressive drugs if necessary to minimize morbidity and mortality.