The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression.

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 CD3CD8 cell levels were higher in patients with PNH. The CD3 cells are positively, correlated with lactate dehydrogenase (LDH; r=0.5453, =0.0040), indirect bilirubin (r=0.4260, =0.0379) and Flear cells in monocytes (r=0.4099, =0.0303). However, a negative correlation was observed between CD3 cells and hemoglobin (r= -0.4530, =0.0105). The total CD19 cells decreased in patients, and CD19 cells were negatively correlated with LDH (r= -0.5640, =0.0077) and Flear cells in monocytes (r= -0.4432, =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, =0.0004), Flear cells in granulocytes (r=0.5357, =0.0396), and monocytes (r=0.6445, =0.0095).

Conclusion: Our results demonstrated that immune abnormalities are associated with PNH development.