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Haemorheology in Gaucher disease

Bax, BE; Richfield, L; Bain, MD; Mehta, AB; Chalmers, RA; Rampling, MW (2005) Haemorheology in Gaucher disease. EUROPEAN JOURNAL OF HAEMATOLOGY, 75 (3). 252 - 258. ISSN 0902-4441 https://doi.org/10.1111/j.1600-0609.2005.00496.x
SGUL Authors: Bax, Bridget Elizabeth

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Abstract

In Gaucher disease, a deficiency of glucocerebrosidase results in the accumulation of glucocerebroside within the lysosomes of the monocyte–macrophage system. Prior to the availability of enzyme replacement therapy (ERT), splenectomy was often indicated for hypersplenism. Haemorheological abnormalities could be expected in view of the anaemia and abnormal lipid metabolism in these patients and the role of the spleen in controlling erythrocyte quality. Objectives: To investigate the effect of Gaucher disease on blood and plasma viscosity, erythrocyte aggregation and erythrocyte deformability, and to determine whether observed rheological differences could be attributed to splenectomy. Methods: Haematological and haemorheological measurements were made on blood collected from 26 spleen-intact patients with Gaucher disease, 16 splenectomised patients with Gaucher disease, 6 otherwise healthy asplenic non-Gaucher disease subjects and 15 healthy controls. Results: No haemorheological differences could be demonstrated between spleen-intact patients with Gaucher disease and the control group. Compared to controls, both asplenic Gaucher disease and asplenic non-Gaucher disease study groups had a reduced MCHC (P = 0.003 and 0.005, respectively) and increased whole blood viscosity at 45% haematocrit (Hct), relative viscosity and red cell aggregation index – all measured at low shear (P < 0.05 for all). Additionally, asplenic patients with Gaucher disease alone showed an increased MCV (P = 0.006), an increased whole blood viscosity at 45% Hct measured at high shear (P = 0.019), and a reduced relative filtration rate (P = 0.0001), compared to controls. Conclusion: These observations demonstrate a direct and measurable haemorheological abnormality in Gaucher disease only revealed when there is no functioning spleen to control erythrocyte quality.

Item Type: Article
Additional Information: This is the accepted version of the following article: Bax, B. E., Richfield, L., Bain, M. D., Mehta, A. B., Chalmers, R. A. and Rampling, M. W. (2005), Haemorheology in Gaucher disease. European Journal of Haematology, 75: 252–258. doi: 10.1111/j.1600-0609.2005.00496.x, which has been published in final form at http://dx.doi.org/10.1111/j.1600-0609.2005.00496.x
Keywords: Case-Control Studies, Erythrocyte Aggregation, Gaucher Disease, Glucosylceramidase, Hemorheology, Humans, Science & Technology, Life Sciences & Biomedicine, Hematology, Gaucher disease, spleen, erythrocytes, whole blood viscosity, plasma viscosity, hemorheology, erythrocyte deformability, erythrocyte aggregability, INHERITED ENZYME DEFICIENCY, REPLACEMENT THERAPY, BLOOD-VISCOSITY, ERYTHROCYTE DEFORMABILITY, CELL AGGREGATION, RISK-FACTORS, GLUCOCEREBROSIDASE, PARAMETERS, MEMBRANE, RHEOLOGY
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cell Sciences (INCCCS)
Journal or Publication Title: EUROPEAN JOURNAL OF HAEMATOLOGY
ISSN: 0902-4441
Related URLs:
Dates:
DateEvent
1 September 2005Published
Web of Science ID: WOS:000231024600011
URI: https://openaccess.sgul.ac.uk/id/eprint/100459
Publisher's version: https://doi.org/10.1111/j.1600-0609.2005.00496.x

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