Expression of blood group i antigen and fetal hemoglobin in paroxysmal nocturnal hemoglobinuria

BACKGROUND: The mechanism by which the paroxysmal nocturnal hemoglobinuria (PNH) clone progressively takes over normal hematopoietic cells remains unknown. The respective in vivo differentiation of normal and PNH erythroid progenitors was investigated through the expression of two fetal erythroid markers (i antigen and fetal hemoglobin [HbF]) whose expression in adult red cells is associated with altered erythropoiesis. STUDY DESIGN AND METHODS: Murine monoclonal antibodies directed against HbF and i and CD59 antigens were used to phenotype red cells of 10 PNH patients. A multiparametric flow cytometry assay of red cells and reticulocytes was designed to assess a possible association of i and HbF with PNH or normal red cells. RESULTS: Most patients exhibited greater expression of i and HbF than did normal controls. In each case, the percentages of i-positive or HbF-positive cells within CD59-deficient and CD59-positive red cells were very close, clearly showing a lack of preferential association of these markers with normal or PNH cells. CONCLUSION: In PNH patients, normal and PNH erythroid progenitors have the same ability to promote HbF and i antigen expression, which suggests that normal and PNH erythroid progenitors (burst-forming units-erythroid, colony-forming units-erythroid, erythroblasts) behave similarly in response to bone marrow stress.