The Studies On The Relationship Between The Expression Of Glycosylphosphatidylinositol-anchored Proteins On Blood Cells And Characters Of Paroxysmal Nocturnal Hemoglobinuria,Aplastic Anemia And Myelodysplastic Syndromes

Objective- To investigate the relationship between paroxysmal nocturnal hemoglobinuria and aplastic anemia, paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes. We assess the express of gly cosy Iphosphatidylinositol-anchored proteins on peripheral erythrocytes and granulocytes in patients with paroxysmal nocturnal hemoglobinuria , aplastic anemia and myelodysplastic syndrome.Methods Flow cytometry(FCM), REDQUANTCD55/CD59 and CELLQUANTCD55/CD59 kits were used to assess peripheral erythrocytes and granulocytes of 42 patients with paroxysmal nocturnal hemoglobinuria,47 patients with aplastic anemia, 36 patients with myelodysplastic syndromes, 4 patients with iron deficiency anemia and 8 patients with autoimmunohemolytic anemia.We also detect WBC, RBC, Hb, Pit, F-Hb, Hp and Ret.Ham test and sucrose test were performced .Results (1) No deficient express were found on erythrocytes and granulocytes in normal volenteers. (2) Deficient expression of glycosylphosphatidylinositol-anchored proteins (CD55 and CD59) were found on erythrocytes and granulocytes in 41 patients with paroxysmal nocturnal hemoglobinuria/aplastic anemia- paroxysmalnocturnal hemoglobinuria syndromes.(3) Deficient express of glycosylphosphatidylinositol-anchored proteins were found on erythrocytes and granulocytes in 16/47(34.04%) patients with aplastic anemia.(4) Deficient express of glycosylphosphatidylinositol -anchored proteins were found on erythrocytes and granulocytes in 5/36(13.89%)patients with myelodysplastic syndrome.Conclusion: Deficient expression of glycosylphosphatidylinositol -anchored proteins (CD55 and CD59) were found on erythrocytes and granulocytes in patients with paroxysmal nocturnal hemoglobinuria/aplastic anemia-paroxysmal nocturnal hemoglobinuria syndromes. A high incidence of GPI-anchorde proteins defect was found in patients with aplastic anemia and with myelodysplastic syndrome. The patients with aplastic anemia and with myelodysplastic syndrome may develop hemoglobinria.