Successful Management of Rituximab Refractory Warm Autoimmune Haemolytic Anaemia with Splenectomy: A Case Report

Abstract

Autoimmune Haemolytic Anaemia (AIHA) is a rare haematological disorder characterised by autoantibodies directed against autologous red blood cells. It can be idiopathic or secondary and classified as Warm type AIHA (WAIHA), cold, or mixed. The primary treatment for WAIHA is a long course of steroid administration, which has an early response rate of 80-90%. However, up to 20-30% of patients require second-line therapy. In the last decade, rituximab has replaced splenectomy as the first-choice therapy for refractory WAIHA patients due to its efficacy and safety. There is a paucity of reported cases of WAIHA refractory to both steroids and rituximab that have responded to splenectomy. This is a case of a 35-year-old female with a history of jaundice and anaemia for the past two years. The patient was diagnosed with WAIHA with a Direct Antibody Test (DAT) positive, IgG positive, and C3d negative results, and massive splenomegaly that was initially refractory to steroids and rituximab but responded to splenectomy. Significant splenomegaly may be an indication for choosing splenectomy over rituximab as a second-line treatment in steroid-refractory WAIHA.

Keywords

• multisection computed tomography
• splenomegaly
• steroid