Fulminant Onset of Systemic Lupus Erythematosus with Mixed Autoimmune Hemolytic Anemia and Sepsis: A Case Report

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BSTRACT

Mixed-type autoimmune hemolytic anemia (AIHA), featuring both warm antibodies and high-titer cold agglutinins, is an exceptionally rare and life-threatening initial presentation of Systemic Lupus Erythematosus (SLE). It poses significant challenges in diagnosis and management, particularly regarding transfusion support. A 25-year-old female presented with severe hemolytic crisis, profound anemia (Hb 4.0 g/dL), and septic shock. Diagnostic workup confirmed mixed-type AIHA with a cold agglutinin titer >1:64 and wide thermal amplitude. The patient’s blood group was O-positive. Immunohematological evaluation, including a monospecific direct antiglobulin test (DAT), revealed strong positivity for both IgG and C3d, and adsorption studies were performed to successfully exclude underlying alloantibodies despite panagglutination. This complex serologic picture caused major crossmatching difficulties, initially delaying compatible transfusion. While awaiting compatible units, the patient received supportive care including oxygen and hemodynamic support. Transfusion was initiated with the least-incompatible, best-matched phenotype-identified units due to critical anemia, with no evidence of transfusion-associated hemolysis. Management was multidisciplinary, involving aggressive antimicrobial therapy for Stenotrophomonas maltophilia sepsis. Immunosuppression was deliberately initiated only after sepsis control was achieved, around day six of hospitalization. This approach led to the resolution of hemolysis and sepsis, with a significant clinical improvement, marked by a steady rise in hemoglobin and normalization of hemolytic markers.