Warm antibody
autoimmune hemolytic anemia (AIHA) results from targeted
antibodies towards the red blood cells (RBCs) and can be either idiopathic or secondary to certain diseases, such as autoimmune disorders or
malignancy, drugs, or
infection. Patients with
DiGeorge syndrome are particularly vulnerable to autoimmune conditions secondary to thymic hypoplasia and dysfunction of the immune system. First-line
therapy for AIHA consists of
corticosteroids, with most patients showing signs of response. Relapses are not uncommon and are treated with
splenectomy or
rituximab. There is a paucity of reports in the literature regarding treatment options beyond this stage. Herein, we describe an unusual case of a 20-year-old female affected by
DiGeorge syndrome with a history of
immune thrombocytopenia (
ITP), who presented with life-threatening AIHA. Standard first- and second-line therapeutic modalities were ineffective in controlling
her disease and she ultimately underwent
plasma exchange therapy with successful resolution of
hemolysis. At her last follow-up, one year after her initial presentation, she remains clinically well without signs of
hemolysis. We conclude that in refractory cases of warm AIHA,
plasma exchange therapy can be a valuable tool in the therapeutic armamentarium.