Publication:

Unusual case of cold agglutination induced hemolysis in a child with systemic lupus erythematosus

Autoimmune hemolytic anemia (AIHA) is a common symptom of systemic lupus erythematosus (SLE), but cold agglutinin disease is uncommon. We describe a rare instance of cold agglutinin causing hemolytic anemia in an eight-year-old girl who had a history of maternal antiphospholipid antibody syndrome. The girl had hemolytic anemia, and the Coombs test revealed both warm and cold antibodies with a significant titer of cold agglutinin. Whole exon sequencing revealed a heterozygous mutation in the GPI gene, which was previously described for non-spherocytic hemolytic anemia, but its significance in SLE is unknown. Patient responded to corticosteroids and azathioprine. The purpose of this article is to highlight this extremely rare SLE manifestation and compare it to previously reported cases.