期刊
BLOOD CELLS MOLECULES AND DISEASES
卷 87, 期 -, 页码 -出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.bcmd.2020.102528
关键词
PNH; GPI-AP deficiency; Platelets; Complement
类别
资金
- Edward P. Evans Foundation
- Henry & Marilyn Taub Foundation [R01HL118281, R01HL123904, R01HL132071, R35HL135795]
- AAMDSIF
- VeloSano Pilot Award
- Vera and Joseph Dresner Foundation-MDS
Platelets and reticulocytes are less sensitive to complement-mediated lysis compared to RBCs but not as resistant as granulocytes. After anti-complement treatment, there is a significant increase in reticulocytes, platelets, RBCs, and granulocytes, supporting the role of PNH hematopoiesis in the context of therapy. Additionally, the study established correlations between sensitivity of PNH cell-types and thrombosis.
While red blood cells (RBCs) and granulocytes have been more studied, platelets and reticulocytes are not commonly used in paroxysmal nocturnal hemoglobinuria (PNH) flow-cytometry and less is known about susceptibility to complement-mediated destruction and effects of anti-complement therapy on these populations. We performed flow-cytometry of RBCs and granulocytes in 90 PNH patients and of platelets and reticulocytes in a subgroup (N = 36), to unveil perturbations of these populations during PNH disease course before and after anti-complement treatment. We found that platelets and reticulocytes were less sensitive to complement-mediated lysis than RBCs but not as resistant as granulocytes, as shown by mean sensitive fraction (difference in a given PNH population vs. PNH granulocyte clone size). In treated patients, reticulocytes, platelets, RBCs (with differences between type II and III) and granulocytes significantly increased post-treatment, confirming the role of PNH hematopoiesis within the context of anti-complement therapy. Moreover, we found that PNH platelet clone size reflects PNH granulocyte clone size. Finally, we established correlations between sensitive fraction of PNH cell-types and thrombosis. In sum, we applied a flow-cytometry panel for investigation of PNH peripheral blood populations' perturbations before and after eculizumab treatment to explore complement-sensitivity and kinetics of these cells during the disease course.
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