Treatment

General

Management of cold haemagglutinin syndromes is difficult. All patients should avoid exposure to cold, and electrically heated gloves and socks are available for use in winter. Wintering in a warm climate is a pleasant alternative. Folic acid supplements should be given to patients with chronic haemolysis.

Alkylating agents

Chlorambucil may be effective in reducing antibody production. Intermittent regimens such as 10 mg/day for 14 days every 4 weeks or continuous treatment of 2-4 mg/day are both effective. Long-term treatment carries the risk of marrow suppression and the development of myelodysplasia and acute myeloid leukaemia. Chlorambucil is most effective when there is an underlying B-cell neoplasm and may be less so in the idiopathic CHAD.

Corticosteroids

Corticosteroids are rarely of use in cold haemagglutinin syndromes. They should be used only in exceptional circumstances when the antibodies are present in low titres and have a high thermal range. Their use should be avoided in other cases.

Splenectomy

Removal of the spleen is rarely of any use. The cells are coated with C3b and destruction occurs mainly in the liver.

Blood transfusion

Blood transfusions should be given with due regard to the difficulty in cross-matching in the presence of cold haemagglutinins. Blood should be given through an in-line blood warmer. The patient should be in a warm environment, preferably at 37°C. Special precautions are needed for surgical procedures to keep the patient warm.

Plasma exchange

The titre of cold agglutinin may be lowered temporarily by plasma exchange. The procedure may be useful in the control of severe symptoms.

Monoclonal antibody therapy

Both rituximab and Campath-IH monoclonal antibodies have been used anecdotally in the treatment of refractory cold-type AIHA. Rituximab is particularly appealing for idiopathic cold haemagglutinin disease and cold agglutinin syndromes with associated B-lymphoproliferative disorders because the antibody targets the CD20 molecule present on mature B-cells, which proliferate in low-grade lymphoproliferative disease, and are the precursors of autoantibody-producing plasma cells in autoimmune cytopenias associated with underlying malignancy as well as in idiopathic autoimmune cytopenia.

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