Paroxysmal Cold Hemoglobinuria Treatment & Management

Updated: Apr 01, 2015
  • Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Medical Care

The mainstay of treatment for paroxysmal cold hemoglobinuria is supportive care and the avoidance of cold exposure.

Once hemolysis is suspected, folic acid 1 mg/d orally should be instituted to help with erythropoiesis. Folic acid is lost via the hemolytic process and hence needs to be replenished.

Administer warmed, packed RBC transfusions for life-threatening hemolysis and symptomatic anemia. Utilizing washed RBC units has not been proven to improve transfusion safety, but this can be performed if patient's condition remains refractory to standard warmed products.

As most of the blood supply is P-antigen positive, finding phenotypic p, also called Tj(a-), blood may not be feasible. However, the antibody should not interfere with donor cell survival, nor should it be problematic with pretransfusion and compatibility testing, as the pathogenic immunoresponse does not occur at normal body temperatures. Treat the uncommon chronic form with RBC transfusions only when severe exacerbation occurs.

Plasma exchange therapy with 5% albumin fluid replacement has been successfully employed. [36] Normal use of plasmapheresis for removal of IgG-induced processes is not as effective due to rebound of immunoglobulin as it shifts from the extravascular to the intravascular compartment. However, due to the low titer and limited production period of the D-L antibody, the process can be effectively controlled. Another theory is that the antibody preferentially binds to the RBC, shifting the antibody equilibrium to the intravascular component, allowing for ease in its removal. [43]

Steroids are commonly employed, but these agents have not been shown to shorten the clinical course of paroxysmal cold hemoglobinuria.

Treat underlying secondary conditions with appropriate medical therapy.

Hydration, alkalinization of the urine, and other measures may become necessary to prevent renal failure. Symptoms of cold urticaria may be ameliorated by antihistamines.

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Surgical Care

Surgery is not indicated in cases of paroxysmal cold hemoglobinuria, other than to aid in the diagnosis of underlying infections or neoplasms.

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Consultations

See the list below:

  • A hematologist-oncologist and an infectious diseases expert may be helpful for the proper diagnosis and treatment of paroxysmal cold hemoglobinuria.
  • May also consult a nephrologist as needed to assist in the care of affected patients.
  • The support of an experienced laboratory/blood bank is essential for all the serologic testing.
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Diet

Folic acid supplements may be useful in the chronic form of paroxysmal cold hemoglobinuria. Encourage patients to eat fresh fruits and vegetables rich in folate.

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Activity

Patients with paroxysmal cold hemoglobinuria should limit activities while severely anemic or if complications such as renal insufficiency are present. Avoid activities in the outdoors that are likely to result in cold exposure. Patients with the chronic form of the disease must wear proper clothes and garments to protect the extremities from becoming chilled.

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