Epstein-Barr Virus-induced Paroxysmal Cold Hemoglobinuria in a 17-Year-old Male

Abstract

Paroxysmal cold hemoglobinuria is a rare autoimmune hemolytic anemia seen almost exclusively in children under 5 years of age after a viral illness. It is mediated by a biphasic polyclonal autoantibody against red blood cells, which causes severe hemolysis that typically self-resolves within 2 weeks without recurrence. While laboratory identification of the aforementioned antibody, the Donath Landsteiner antibody, would confirm this diagnosis, a negative test does not rule out this condition in the appropriate clinical context. We report on the rare occurrence of a severe presentation of paroxysmal cold hemoglobinuria in a 17-year-old male with Epstein-Barr virus infection.

Challenges in recognising paroxysmal cold hemoglobinuria

“…8 While the authors report that biphasic hemolysis was present in the DL test, it would have been informative to know whether hemolysis was also observed in the patient samples maintained at cold and/or warm temperature, as this would give credence to the presence of a clinically significant cold and/or warm autoantibody. Of note, a negative DL test does not necessarily exclude a diagnosis of PCH in the appropriate clinical context 9 ; low and undetectable DL antibody titers are the most common cause of false-negative results. 8 Methods to enhance the detectability of these low-titre antibodies include enzyme treatment of reagent RBCs, which will increase the exposure of the P antigen, thereby potentially allowing for heightened binding of the DL antibody.…”