Case Report: Auto Anti-D, Auto-IgG, and Cold Agglutinins in a Rh(D) Positive Patient with CD5-Negative B Lymphoproliferative Disorder Causing Autoimmune Haemolytic Anaemia: A Single-Centre Experience

Abstract

Background: Autoimmune hemolytic anemia (AIHA) is defined by premature red blood cell destruction mediated by autoantibodies against self-antigens. Autoantibodies with specificity to the D antigen (auto-anti-D) are exceedingly uncommon. Their presence creates substantial diagnostic and transfusion-related challenges, particularly in patients with prior transfusion exposure or partial D variants, where differentiation between auto anti-D and allo anti-D is problematic. This distinction is clinically critical, as transfusion of Rh(D)-positive red cells in patients with unrecognized allo anti-D may precipitate severe hemolytic transfusion reactions.

Case Presentation: We describe a Rh(D)-positive male with a CD5-negative lymphoproliferative disorder who experienced a relapse of AIHA, having a prior history of auto-IgG–mediated hemolysis. The patient presented with typical manifestations of hemolysis, including fatigue, dyspnoea, and transfusion-refractory anemia. Notably, serological evaluation revealed the emergence of auto-anti-D antibodies in a Rh(D)-positive individual—an infrequent finding that may reflect immune dysregulation associated with disease progression. The coexistence of auto-IgG and newly detected cold agglutinins further underscores the complex immunopathogenesis of AIHA in this setting.

Conclusion: Although auto-anti-D antibodies are often regarded as having limited clinical significance, their presence alongside other autoantibodies may intensify hemolysis and worsen clinical outcomes. Awareness and accurate identification of this rare antibody profile are essential for optimizing transfusion strategies and guiding overall disease management.