Density, heterogeneity and deformability of red cells as markers of clinical severity in hereditary spherocytosis

• Published in: Haematologica (Roma) Vol. 105; no. 2; pp. 338 - 347
• Main Authors: Huisjes, Rick, Makhro, Asya, Llaudet-Planas, Esther, Hertz, Laura, Petkova-Kirova, Polina, Verhagen, Liesbeth P., Pignatelli, Silvia, Rab, Minke A.E., Schiffelers, Raymond M., Seiler, Elena, van Solinge, Wouter W., Corrons, Joan-LLuis Vives, Kaestner, Lars, Mañú-Pereira, Maria, Bogdanova, Anna, van Wijk, Richard
• Format: Journal Article
• Language: English
• Published: Italy Ferrata Storti Foundation 01.02.2020
• Subjects: Ankyrins; Erythrocyte Membrane; Erythrocytes; Humans; Reticulocyte Count; Spherocytosis, Hereditary - diagnosis

Hereditary spherocytosis (HS) originates from defective anchoring of the cytoskeletal network to the transmembrane protein complexes of the red blood cell (RBC). Red cells in HS are characterized by membrane instability and reduced deformability and there is marked heterogeneity in disease severity...