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- W4283369782 abstract "Background: Defective spleen function (hyposplenism) affects subjects with sickle cell disease (SCD) and is associated with complications. Red blood cell (RBC) alterations are very accurate markers of spleen function. Vacuole-containing RBC (pocked RBC) circulate in high proportions (10 to 70%) in the blood of hyposplenic subjects while their proportion does not exceed 5% of RBC in healthy subjects. Vacuoles in pocked RBC are generally considered as remnants of organelles in erythroblasts. Aims: The aim is to determine the origin of vacuoles Methods: Patients with SCD who benefit from an exchange transfusion program, have not only their own HbSS RBC but also transfused HbAA RBC in circulation. In vitro sickling “Emmel test” discriminates HbSS RBC that sickle, from HbAA RBC that do not sickle. We perfused a mixture of HbSS RBC and HbAA RBC (from exchange transfusion left-overs) into human spleens ex-vivo. We then analyzed spleen sections and quantified vacuoles in RBC containing hemoglobin polymers or not. Results: Using Emmel Test, we observed vacuoles in HbSS RBC but also in transfused HbAA RBC. Vacuoles in HbAA RBC were more frequent in transfused SCD patients than in healthy subjects (25.1±4.41% vs. 2.8±2.1%). In our experimental ex vivo perfusion, most RBC without hemoglobin polymers is HbAA RBC. We observed a high rate of vacuoles in both polymerized and non-polymerized RBC (29.8±3.7% vs. 17.9±5.8%). Summary/Conclusion: Taken together, these results strongly suggest that vacuoles are created de novo in transfused HbAA RBC. Counting vacuoles in RBC labeled with anti-HbS and anti-HbA antibody is ongoing to validate these observations. When the spleen is dysfunctional, these pocked RBC stay in circulation hence their validity as a spleen function marker. De novo appearance of vacuoles in transfused HbAA RBC likely impacts the kinetics of reappearance of pocked RBC in circulation following regular or exchange transfusion of hyposplenic subjects, and opens interesting prospects on cell processes involved in their genesis." @default.
- W4283369782 created "2022-06-25" @default.
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- W4283369782 date "2022-06-01" @default.
- W4283369782 modified "2023-10-01" @default.
- W4283369782 title "PB2216: VACUOLES APPEAR IN RED BLOOD CELLS TRANSFUSED TO PATIENTS WITH SICKLE CELL DISEASE-RELATED HYPOSPLENISM" @default.
- W4283369782 doi "https://doi.org/10.1097/01.hs9.0000851692.16423.82" @default.
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