Fatal, Rapidly Progressive EBV-Associated Warm Autoantibody Hemolytic Anemia (WAIHA): A Rare Case Study

Abstract Introduction/Objective Separately WAIHA and Epstein-Barr Virus-infectious mononucleosis (EBV-IM) are well- studied clinical entities. EBV-IM cases are commonly linked with cold autoagglutinins (IgMs with anti-i specificity), but uncommonly associated with WAIHA. More rarely, EBV-IM can be associated with rapidly fatal AIHA. Prompt and appropriate diagnostic Blood Bank testing for WAIHA in EBV-infected patients should include work-ups to prevent missing the possibility of treating complicating WAIHA. Methods/Case Report A 24-year-old woman presented to the ED with a month of cough/cold symptoms, night sweats, cervical lymphadenopathy, and imaging showing diffuse thoracic lymphadenopathy. DNA/PCR testing was positive for active EBV infection and a lymph node biopsy was diagnostic for EBV lymphadenitis. Increasing respiratory distress developed, coupled with renal and liver insufficiency, hypotension, and possible DIC, and (suspected septic) shock. On hospital day (HD) 5, altered mental status worsened, cardiac arrest occurred requiring resuscitation and vasopressors, and acidosis trended rapidly higher. The patient rapidly decompensated and she was compassionately extubated, expiring on HD 6. There were significant laboratory interval changes in the final 20 hours of life. New positive DATs (with anti-IgG and - C3b/d), a pan-reactive warm autoantibody of broad specificity (in plasma & eluate), onset of acute hemolysis (nadir Hgb to 4.6 g/dL with net drop of 4.7 g/dL & LDH 23, 582 U/L), coagulopathy (peak PT 96.6 sec) and evidnece of organ failure. Blood Bank testing, received on HD 5 at 21:45, was resulted in the EMR on HD 6 at 00:30 and the patient expired at 05:15--further illustrating the rapid clinical course presented in this case. Results (if a Case Study enter NA) N/A Conclusion This patient died of complications directly associated with acute, immune-mediated intravascular hemolysis leading to high-output cardiac failure and resulting in multi-organ failure. With a relative paucity of published EBV-IM WAIHA associations, the rapidly evolving unexpected hematologic sequelae observed complicated the clinical recognition and treatment of severe WAIHA during the course of treatment for clinically suspected DIC. The rapid progression of this patients’ symptoms and laboratory values did not allow for adequate recognition of WAIHA-related pathogenesis and appropriate interventions.