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 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.
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