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Sickle cell anaemia: haemorheological aspects
Maria Cristina Martorana1, Giorgio Mojoli, Paolo Cianciulli
1Centro Aziendale Produzione Emocomponenti (CAPE), Azienda Ospedaliera San Camillo-Forlanini, Via Ramazzini,15 00151 Rome, Italy. mmartorana@scamilloforlanini.rm.it
Annali Dell'Istituto Superiore Di Sanita
|July 20, 2007
Summary
Sickle cell disease (SCD) impairs red blood cell flexibility due to hemoglobin S polymers. This study reveals altered viscoelastic properties in sickle cells, with transfusions reducing variability in patients dependent on them.
Area of Science:
- Hematology
- Biophysics
- Rheology
Background:
- Erythrocyte shape and membrane integrity depend on deformability and permeability, factors often overlooked in standard lab tests.
- Red blood cell membrane stiffness, geometry, and viscoelasticity influence cell survival and function.
- Sickle cell disease (SCD) significantly impairs red blood cell flexibility due to intracellular sickle hemoglobin (Hb S) polymers, leading to critical rheologic alterations.
Purpose of the Study:
- To investigate the rheologic behavior of sickle cells from an original perspective.
- To evaluate the viscoelastic properties of sickle cells in SCD patients using an oscillating harmonic sinusoidal mode.
- To compare rheologic properties between SCD patients with (TD) and without (NTD) transfusion dependence.
Main Methods:
- Evaluation of viscoelastic properties of sickle cells in an oscillating harmonic sinusoidal mode.
- Comparison of rheologic behavior between transfusion-dependent (TD) and non-transfusion-dependent (NTD) SCD patients.
- Analysis of red blood cell behavior in SCD from a rheological standpoint.
Main Results:
- The study confirmed significant rheologic impairment in sickle cell (SC) blood.
- Sickle cells exhibit altered viscoelastic properties due to Hb S polymerization and sol-gel transitions.
- Transfusion-dependent (TD) patients displayed less heterogeneity in rheologic behavior compared to non-transfusion-dependent (NTD) patients, attributed to transfusion effects.
Conclusions:
- The rheologic impairment in sickle cell disease is confirmed.
- Transfusions appear to normalize sickle cell rheology, reducing variability in TD patients.
- Analysis of viscoelastic properties offers a valuable tool for monitoring SCD treatments, including transfusions and pharmacological interventions.
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