[HTML][HTML] Metabolic fingerprint in hereditary spherocytosis correlates with red blood cell characteristics and clinical severity
B van Dooijeweert, MH Broeks, NM Verhoeven-Duif… - …, 2021 - journals.lww.com
Hereditary spherocytosis (HS) is the most common cause of hereditary chronic hemolytic
anemia in people of northern European descent (1: 2000–3000). 1 Characterized by
impaired red blood cell (RBC) membrane integrity due to disruption of the (vertical)
association between the cytoskeleton and the plasma membrane, RBCs from HS patients
show enhanced membrane loss and thereby surface area. As a result RBCs become
spheroidal with decreased deformability, leading to premature splenic sequestration. 2 HS is …
anemia in people of northern European descent (1: 2000–3000). 1 Characterized by
impaired red blood cell (RBC) membrane integrity due to disruption of the (vertical)
association between the cytoskeleton and the plasma membrane, RBCs from HS patients
show enhanced membrane loss and thereby surface area. As a result RBCs become
spheroidal with decreased deformability, leading to premature splenic sequestration. 2 HS is …