Paroxysmal Cold Hemoglobinuria (PCH) is characterized
by a sudden onset of hemoglobinuria either spontaneously or after exposure
to cold. It is considered as one type of Cryopathic Hemolytic Syndrome,
which includes two types of autoimmune hemolytic anemias because of
Donath-Landsteiner Syndrome; Cryopathic Hemolytic
Accounts for up to 5% of autoimmune hemolytic anemia.
It may be responsible for more than 40% of autoimmune hemolytic anemias
in children younger than 5 years. There is no race and sex predilection.
Infections, both bacterial and viral, induce the
formation of “Donald-Landsteiner” antibodies that are specific for the
human P blood group antigen. The P antigen is a glycosphingolipid that is similar to
glycolipids in many microorganisms. The Donald-Landsteiner autoantibody is a biphasic hemolysin capable of causing severe hemolysis. These antibodies bind to red cells upon
exposure to cold and dissociate at 37°C (98.6°F). However, prior to
dissociation, they fix complement and cause hemolysis. Why infections stimulate these
antibodies is unknown. During the early 1900s, syphilis was the leading etiology,
although today it is more commonly viral.
The biphasic Donald-Landsteiner test, which consists of
mixing the patient's serum with RBCs at 4°C (39.2°F), then heating
them to 37°C (98.6°F) and assessing for hemolysis.
Fatigue, dyspnea, jaundice, dark urine, and cold
urticaria are frequent. Usually self-limited, although occasional fulminant
hemolysis requires transfusion. The acute transient form is more common in children than adults.
Check hematocrit. Check blood bank
availability of P antigen-negative. Transfusion of P-positive RBCs is usually
necessary, as the incidence of P antigen-positive blood is about 1:200,000 in general
population but can be done safely.
Avoid hypothermia. Use fluid and blood
warmers. Use P antigen-negative blood if available, though P-positive blood is acceptable in
Jeffries LC: Transfusion therapy in autoimmune hemolytic anemia. Hematol Oncol Clin North Am