PT  - JOURNAL ARTICLE
AU  - Wang, Guanrou
AU  - Che, Mengting
AU  - Zeng, Lijie
AU  - Liu, Hui
AU  - Li, Liyan
AU  - Liu, Zhaoyun
AU  - Fu, Rong
TI  - The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression
AID  - 10.15537/smj.2024.45.4.20231010
DP  - 2024 Apr 01
TA  - Saudi Medical Journal
PG  - 424--432
VI  - 45
IP  - 4
4099  - http://smj.org.sa/content/45/4/424.short
4100  - http://smj.org.sa/content/45/4/424.full
SO  - Saudi Med J2024 Apr 01; 45
AB  - 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.