Case Presentation: A 21-year-old female with history of IgG immune deficiency requiring intravenous immunoglobulin transfusions from the age of 12-17 presented to our service with one-week complaint of dizziness and fatigue. At presentation, she reported dyspnea, chest pain, intermittent palpitations, and nausea. She had had a brief pre-syncopal episode and an episode of nausea and vomiting a few days prior. In the emergency department, she was found to have a heart rate of 117 beats/min, respiratory rate of 25 breaths/min, pallor, and scleral icterus. She denied history of blood transfusions, anemia, liver disease, alcohol use, tobacco use, hematuria, or easy bruising. She denied any sexually transmitted diseases or intravenous drug use.
Laboratory findings were concerning for a warm hemolytic anemia including a white blood cell count of 24,000 cells/UL, hemoglobin of 5.9 g/dL, hematocrit of 18%, total bilirubin of 5.7 mg/dL with 4.9 mg/dL indirect bilirubin, lactate dehydrogenase of 1200 U/L, and haptoglobin less than 8 mg/dL. Peripheral blood smear showed normochromic normocytic anemia with prominent spherocytes. She had a positive direct antiglobulin test. Infectious work-up was positive for Epstein Bar Virus (EBV) IGG and IGM antibodies. Our patient was treated with intravenous immunoglobulin and high dose glucocorticoids with improvement of her symptoms and anemia. She was discharged on steroid taper with hematology follow up.
Discussion: Autoimmune Hemolytic Anemia (AIHA) is a rare disease with a prevalence of 1-3/100,000 and is characterized by the production of autoantibodies against physiologic antigens found on red blood cells. The most common form of AIHA is due to warm reactive antibodies. In warm antibody AIHA, the current first line treatment is immunosuppressive drugs including glucocorticoids. AIHA can be seen in the context of Combined Variable Immune Deficiency (CVID) as in our patient, thought to be secondary to dysregulated immunity with an estimated total incidence of 6%. Interestingly, AIHA presents with an equal incidence both pre- and post-immunoglobulin replacement therapy.
Conclusions: While our patient had lab work consistent with recent EBV infection, this infection is more commonly associated with cold hemolytic anemia. Therefore, it is more likely that this case demonstrates a clinical correlate of symptomatic autoimmune hemolytic anemia that presented distant to the initial onset and treatment of CVID. Moreover, this case highlights the importance of a thorough history for patients presenting with possible hemolysis as there is increased risk for AIHA in patients with known CVID, and emergent initiation of high-dose steroids can be life-saving.