• Saudi Med J · Apr 2024

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

    • Guanrou Wang, Mengting Che, Lijie Zeng, Hui Liu, Liyan Li, Zhaoyun Liu, and Rong Fu.
    • From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People's Republic of China.
    • Saudi Med J. 2024 Apr 1; 45 (4): 424432424-432.

    ObjectivesTo suggest the presence of a hyperimmune state in patients, and indicate that immune system attack on glycosylphosphatidylinositol (+) (GPI+) cells while escaping GPI- cell immunity.MethodsWe 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.ResultsThe 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).ConclusionOur results demonstrated that immune abnormalities are associated with PNH development.Copyright: © Saudi Medical Journal.

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