The thalassaemia syndromes / D.J. Weatherall.

  • David Weatherall
Date:
1972
Catalogue details

Licence: Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)

Credit: The thalassaemia syndromes / D.J. Weatherall. Source: Wellcome Collection.

    291/392 page 277
    THE MANAGEMENT OF THALASSAEMIA 277 The severity may certainly be modified by the use of washed cells, dextran sedimentation or filters to remove the white cells. Suitable filters may be obtained from Baxter Laboratories, U.S.A. In the authors' experi¬ ence pyrexial reactions can be ameliorated in some cases by covering the period of the blood transfusion with corticosteroid therapy. A promising approach to the avoidance of pyrexial reactions and to shortening the time required for each transfusion is the use of frozen red cells (Korn and Stewart, 1971). Dr J.W.Stewart (personal communication, 1971) has treated two thalassaemic children with frozen red cell transfusions every seven weeks for an eighteen month period. The transfusion intervals have been longer than with ordinary packed red cell transfusions and there have been no pyrexial reactions. The low incidence of pyrexial and ana¬ phylactoid reactions following the use of frozen red cell transfusions is probably because most of the plasma proteins and leucocytes are remxoved prior to freezing. This technique obviously requires further investigation but may greatly reduce the incidence of non-specific transfusion reactions in thalassaemic children on long term transfusion therapy. Sensitisation against one or more minor blood group antigens is a fairly common occurrence in patients receiving multiple blood transfusions. After the Rh antigen, D, the red cell antigens most likely to sensitise the multitransfused recipient are C, c, E, Kell and Fy^. The importance of accurate genotyping before the transfusion programme is started has already been emphasised. Whenever possible blood of identical genotype should be transfused. Once antibodies are present it is necessary to find a compatible donor, which may be extremely difficult, and some help may be obtained by covering the period of transfusion with corticosteroid therapy. A complication of this type should be suspected when the gaps between blood transfusion requirements become much shorter. These problems, and the diagnosis and management of the incompatible transfusion, have been fully discussed by Mollison (1967). Other dangers of blood transfusion include those of passage of infection, particularly serum hepatitis, syphilis and malaria, and careful screening of donors for malaria in tropical countries is of paramount importance. It is Hkely that fresh blood may be a source of the cytomegalovirus (CMV) and thus blood less than 48 hr old should not be used unless the donor is sero-negative for the CM virus. It is probable that screening for the SH (Australia) antigen will provide a method of preventing serum hepatitis in the near future (Turner, 1971) and certainly priority should be given to screening donors for thalassaemic children in this way.
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