Abstract
OBJECTIVES: To suggest the presence of a hyperimmune state in patients, and indicate that immune system attack on glycosylphosphatidylinositol (+) (GPI(+)) cells while escaping GPI(-) cell immunity. METHODS: We retrospective the immune cell subtypes in peripheral blood from 25 patients visiting Tianjin Medical University General Hospital, Tianjin, China, with classical paroxysmal nocturnal hemoglobinuria (PNH) and 50 healthy controls. RESULTS: The total CD3(+) and CD3(+)CD8(+) cell levels were higher in patients with PNH. The CD3(+) cells are positively, correlated with lactate dehydrogenase (LDH; r=0.5453, p=0.0040), indirect bilirubin (r=0.4260, p=0.0379) and Flear(-) cells in monocytes (r=0.4099, p=0.0303). However, a negative correlation was observed between CD3(+) cells and hemoglobin (r= -0.4530, p=0.0105). The total CD19(+) cells decreased in patients, and CD19(+) cells were negatively correlated with LDH (r= -0.5640, p=0.0077) and Flear(-) cells in monocytes (r= -0.4432, p=0.0341). Patients showed an increased proportion of total dendritic cells (DCs), with a higher proportion of myeloid DCs (mDCs) within the DC population. Moreover, the proportion of mDC/DC was positively correlated with CD59(-) cells (II + III types) in red cells (r=0.7941, p=0.0004), Flear(-) cells in granulocytes (r=0.5357, p=0.0396), and monocytes (r=0.6445, p=0.0095). CONCLUSION: Our results demonstrated that immune abnormalities are associated with PNH development.