Abstract
BACKGROUND Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic disease caused by the Donath-Landsteiner (DL) antibody. Paroxysmal nocturnal hemoglobinuria (PNH) is a non-autoimmune hemolytic disease that is caused by a dysfunction in the synthesis of the glycosyl phosphatidylinositol anchor protein, resulting in the deregulation of the complement cascade and hypersensitivity for a hemolytic attack against erythrocytes. The mechanisms of these 2 hemolytic diseases are distinct. If PCH and PNH coexist in a patient, it is difficult to perform a differential diagnosis. We introduce a case of PCH that had DL antibodies and large PNH-type clones. CASE REPORT An 82-year-old female patient was referred to our hospital because of anemia. Initial workup revealed a negative antiglobulin test and a positive DL test. For the differential diagnosis, we surveyed the population of cells that had PNH-type clones, which revealed erythrocyte PNH clones (19.6%) and granulocyte PNH clones (73.3%). During the patient's clinical course, mild hemolysis persisted without any attack. The percentage of the PNH-type erythrocytes was not obviously changed, and the DL antibody was detected 8 months after the initial admission. We determined that the persistent mild anemia was caused by concomitant diseases of PCH and PNH, although determining which of the 2 hemolytic systems was primarily responsible for the anemia was difficult. CONCLUSIONS When considering the differential diagnosis for hemolytic diseases, an adequate combination of laboratory tests for hemolysis is required.