Successful Treatment of Warm Autoimmune Hemolytic Anemia with a Positive Donath-Landsteiner Test Using Rituximab

Abstract

Paroxysmal cold hemoglobinuria (PCH) is a rare disease in adults, and its concurrent presentation with warm-type autoimmune hemolytic anemia (AIHA) has not yet been reported. We encountered a 19-year-old woman with AIHA and a positive Donath-Landsteiner test result identified by a hemolytic attack during blood transfusion. She also showed positive results for the direct Coombs and Donath-Landsteiner antibody tests. After treatment with prednisolone followed by rituximab, the AIHA improved, and the Donath-Landsteiner antibody test result turned negative. Clinicians should be aware that patients may present with concurrent warm-type AIHA and PCH and consider rituximab for its treatment.

Autoimmune Hemolytic Anemias: Classifications, Pathophysiology, Diagnoses and Management

“…Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia that is defined by the destruction of red blood cells in the blood vessels due to the activation of the complement system [2,3,[94][95][96][97][98][99][100][101][102][103][104]. This destruction happens when the body is exposed to cold temperatures [3,12,100].…”

“…PCH in children is usually self-remitting. In severe cases requiring transfusion, approaches that have been successfully described include plasma exchange, rituximab, and complement inhibitors such as eculizumab [3,97,[101][102][103][104].…”

“…Approximately 1% to 10% of people diagnosed with AIHA have a negative DAT [3,103,104]. The DAT is a diagnostic technique that has a sensitivity range from 92% to 97% for detecting AIHA.…”