Paroxsymal Cold Hemoglobinuria (PCH)

What is PCH?

        Autoantibody (Biphasic hemolysin) that binds to Red Blood Cells after the exposure to the Cold, and then the Red Blood Cells lyse and release Hemoglobin when body is warmed back up to body temperature.

Clinical Presentation:

         Recurrence of a fever and Dark urine 1-2 weeks after a Urinary Tract Infection (UTI)

         Can occur after the exposure to the Cold

         Hemolysis is usually acute and severe

              Hemoglobin < 5 g/dl

 

         Symptoms:

              Fever

              Abdominal Pain

              Hepatosplenomegaly (Enlarged Liver and Spleen)

              Fatigue

              Hemoglobinuria (dark urine; hemoglobin in the urine)

              Pallor

              Jaundice

 

Occurence:

    Occurs more often in children after viral infection but spontaneously resolves.

                Adults are more chronic, but not as severe

 

Diagnosis:

               Identification of symptoms and lab values

               Diagnostic Lab Test: Donath-Landsteiner Test

                            Patient plasma and Cells are incubated at Cold and Warm Temperatures.

                                             If Hemolysis appears in the tubes incubated first at 4C and then at 37C and not in the other tubes, it is Positive.

 

Antibody Specificity:

              The autoantibody is usually IgG with anti-P specificity and is a biphasic hemolysin.

 

Treatment:

             Supportive Therapy through blood transfusion and medications