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Article

The Erythrocyte Sedimentation Rate and Its Relation to Cell Shape and Rigidity of Red Blood Cells from Chorea-Acanthocytosis Patients in an Off-Label Treatment with Dasatinib

1
Theoretical Medicine and Biosciences, Saarland University, 66424 Homburg, Germany
2
Experimental Physics, Saarland University, 66123 Saarbruecken, Germany
3
Translational Neurodegeneration Section “Albrecht-Kossel”, Department of Neurology, University Medical Center Rostock, University of Rostock, 18051 Rostock, Germany
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Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany
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Institute for Clinical and Experimental Surgery, Saarland University, 66424 Homburg, Germany
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Institute of Biological Information Processing and Institute for Advanced Simulation, Forschungszentrum Jülich, 52425 Jülich, Germany
7
Central Laboratory, Saarland University Medical Centre, 66424 Homburg, Germany
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Cysmic GmbH, 66123 Saarbrücken, Germany
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Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universität, 81366 Munich, Germany
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Physics and Materials Science Research Unit, University of Luxembourg, 1511 Luxembourg, Luxembourg
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DZNE, German Center for Neurodegenerative Diseases, Research Site Rostock/Greifswald, 18051 Rostock, Germany
12
Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Center Rostock, University of Rostock, 18051 Rostock, Germany
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Academic Editor: Gregory Barshtein
Biomolecules 2021, 11(5), 727; https://doi.org/10.3390/biom11050727
Received: 20 April 2021 / Revised: 7 May 2021 / Accepted: 10 May 2021 / Published: 12 May 2021
(This article belongs to the Special Issue Biochemical and Biophysical Properties of Red Blood Cells in Disease)
Background: Chorea-acanthocytosis (ChAc) is a rare hereditary neurodegenerative disease with deformed red blood cells (RBCs), so-called acanthocytes, as a typical marker of the disease. Erythrocyte sedimentation rate (ESR) was recently proposed as a diagnostic biomarker. To date, there is no treatment option for affected patients, but promising therapy candidates, such as dasatinib, a Lyn-kinase inhibitor, have been identified. Methods: RBCs of two ChAc patients during and after dasatinib treatment were characterized by the ESR, clinical hematology parameters and the 3D shape classification in stasis based on an artificial neural network. Furthermore, mathematical modeling was performed to understand the contribution of cell morphology and cell rigidity to the ESR. Microfluidic measurements were used to compare the RBC rigidity between ChAc patients and healthy controls. Results: The mechano-morphological characterization of RBCs from two ChAc patients in an off-label treatment with dasatinib revealed differences in the ESR and the acanthocyte count during and after the treatment period, which could not directly be related to each other. Clinical hematology parameters were in the normal range. Mathematical modeling indicated that RBC rigidity is more important for delayed ESR than cell shape. Microfluidic experiments confirmed a higher rigidity in the normocytes of ChAc patients compared to healthy controls. Conclusions: The results increase our understanding of the role of acanthocytes and their associated properties in the ESR, but the data are too sparse to answer the question of whether the ESR is a suitable biomarker for treatment success, whereas a correlation between hematological and neuronal phenotype is still subject to verification. View Full-Text
Keywords: acanthocytes; dasatinib; erythrocyte sedimentation rate; 3D shapes; microfluidics; phase diagram; cell rigidity; mesoscopic modeling acanthocytes; dasatinib; erythrocyte sedimentation rate; 3D shapes; microfluidics; phase diagram; cell rigidity; mesoscopic modeling
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MDPI and ACS Style

Rabe, A.; Kihm, A.; Darras, A.; Peikert, K.; Simionato, G.; Dasanna, A.K.; Glaß, H.; Geisel, J.; Quint, S.; Danek, A.; Wagner, C.; Fedosov, D.A.; Hermann, A.; Kaestner, L. The Erythrocyte Sedimentation Rate and Its Relation to Cell Shape and Rigidity of Red Blood Cells from Chorea-Acanthocytosis Patients in an Off-Label Treatment with Dasatinib. Biomolecules 2021, 11, 727. https://doi.org/10.3390/biom11050727

AMA Style

Rabe A, Kihm A, Darras A, Peikert K, Simionato G, Dasanna AK, Glaß H, Geisel J, Quint S, Danek A, Wagner C, Fedosov DA, Hermann A, Kaestner L. The Erythrocyte Sedimentation Rate and Its Relation to Cell Shape and Rigidity of Red Blood Cells from Chorea-Acanthocytosis Patients in an Off-Label Treatment with Dasatinib. Biomolecules. 2021; 11(5):727. https://doi.org/10.3390/biom11050727

Chicago/Turabian Style

Rabe, Antonia, Alexander Kihm, Alexis Darras, Kevin Peikert, Greta Simionato, Anil K. Dasanna, Hannes Glaß, Jürgen Geisel, Stephan Quint, Adrian Danek, Christian Wagner, Dmitry A. Fedosov, Andreas Hermann, and Lars Kaestner. 2021. "The Erythrocyte Sedimentation Rate and Its Relation to Cell Shape and Rigidity of Red Blood Cells from Chorea-Acanthocytosis Patients in an Off-Label Treatment with Dasatinib" Biomolecules 11, no. 5: 727. https://doi.org/10.3390/biom11050727

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