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Haemorheology in Gaucher disease
Bridget E Bax1, Linda Richfield, Murray D Bain
1Child Health, Department of Clinical Developmental Sciences, St George's, University of London, UK. bebax@sgul.ac.uk
European Journal of Haematology
|August 18, 2005
Summary
Gaucher disease causes abnormal blood flow (haemorheology) but only when the spleen is absent. Without a functioning spleen, patients show reduced red blood cell quality and increased blood viscosity.
Area of Science:
- Hematology
- Rheology
- Gaucher Disease Pathophysiology
Background:
- Gaucher disease involves glucocerebrosidase deficiency, leading to glucocerebroside accumulation.
- Splenectomy was a common treatment for hypersplenism in Gaucher disease before enzyme replacement therapy (ERT).
- Haemorheological abnormalities are anticipated due to anemia, altered lipid metabolism, and the spleen's role in erythrocyte quality control.
Purpose of the Study:
- To investigate the impact of Gaucher disease on blood viscosity, plasma viscosity, erythrocyte aggregation, and erythrocyte deformability.
- To determine if splenectomy influences these observed rheological differences.
Main Methods:
- Hematological and haemorheological measurements were performed on blood samples.
- Study groups included spleen-intact Gaucher patients, splenectomized Gaucher patients, asplenic non-Gaucher subjects, and healthy controls.
Main Results:
- No significant haemorheological differences were found between spleen-intact Gaucher patients and controls.
- Asplenic patients (both Gaucher and non-Gaucher) showed reduced MCHC and increased whole blood viscosity, relative viscosity, and red cell aggregation index compared to controls.
- Asplenic Gaucher patients additionally exhibited increased MCV, higher whole blood viscosity at high shear, and reduced relative filtration rate.
Conclusions:
- Haemorheological abnormalities in Gaucher disease are directly measurable and evident only in the absence of a functioning spleen.
- The spleen plays a crucial role in maintaining normal erythrocyte quality and blood rheology in Gaucher disease patients.