Paroxysmal Cold Hemoglobinuria

At a glance

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.

Synonyms

Donath-Landsteiner Syndrome; Cryopathic Hemolytic Syndrome.

Incidence

Accounts for up to 5% of autoimmune hemolytic anemia, which occur between 1:35,000 and 1:80,000. 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.

Genetic inheritance

None.

Pathophysiology

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 or bacterial infection such as measles, mumps, cytomegalovirus, Epstein-Barr virus, varicella, influenza, mycoplasma, and Haemophilus influenzae.

Diagnosis

Present as normocytic or macrocytic anemia. The biphasic Donald-Landsteiner test, which consists of mixing the patient’s serum with red blood cells (RBCs) at 4°C (39.2°F), then heating them to 37°C (98.6°F) and assessing for hemolysis is used for diagnosis.

Clinical aspects

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. Immunosuppressive or pharmacologic drug therapy is rarely required.

Precautions before anesthesia

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.

Anesthetic considerations

Avoid hypothermia. Use fluid and blood warmers. Use P antigen-negative blood if available, though P-positive blood is acceptable in urgent situations.

Pharmacological Implications

There is no known implication.

References