A patient with sickle cell disease may have a hemolytic transfusion reaction following red blood cell transfusion. This appears to be due to peripheral consumption of the red cells by macrophages. Recognition is important since additional transfusion therapy can be fatal.


Clinical features:

(1) The hemoglobin in the post-transfusion specimen is less than that in the pre-transfusion sample, with other signs of hemolysis (jaundice, decreased haptoglobin).

(2) The hemolysis does not appear to be related binding of allo- or autoantbodies to red cell antigens. Some patients show HLA antibodies.

(3) Marked reticulocytopenia.

(4) Recurrence of the hemolysis with additional transfusion of red blood cell products, with possible renal failure, shock and death.


Management involves the administration of:

(1) intravenous immune globulin concentrates (IVIG)

(2) corticosteroids

(3) avoidance of additional red blood cell product administration.


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