Transfusion-related acute lung injury due to granulocyte-agglutinating antibody in a patient with paroxysmal nocturnal hemoglobinuria

BACKGROUND: Transfusion-related acute lung injury (TRALI) is usually report ed after the transfusion of blood components from donors with white cell (W BC) antibodies, but only very rarely if the patient has these antibodies. T he pathogenesis of TRALI is not fully understood. Not all transfusion recip ients develop TRALI, even though WBC antibodies are present in the donor or the recipient. CASE REPORT: A patient with paroxysmal nocturnal hemoglobinuria (PNH) who d eveloped TRALI after the transfusion of non-WBC-reduced red cells is descri bed. Granulocyte-agglutinating anti-5b was detected in his serum, and the c rossmatch with the donor granulocytes was positive. The patient also develo ped a severe exacerbation of hemolysis with renal failure; serologic result s excluded an immune hemolytic posttransfusion reaction. The patient recove red from both events after about 1 week. CONCLUSION: Granulocyte-agglutinating antibodies present in the recipient p lay an important role in TRALI, and also other factors may contribute to it s pathogenesis. The reaction between the PNH patient's antibody (anti-5b) a nd transfused WBCs was found not only to be responsible for the respiratory distress but also to have triggered, through the innocent-bystander mechan ism of complement activation, an intensive hemolysis, which was very likely a contributing factor in the development of TRALI.