Review Article

Role of Extracellular Hemoglobin in Thrombosis and Vascular Occlusion in Patients with Sickle Cell Anemia

Figure 1

Hb-bound VWF multimers are prevalent in SCD patients and are hyperactive. (a): the VWF was purified from plasma (1 mL) of SCD patients or normal individuals by a Superose gel filtration column. The 1st protein peak (at 280 nm UV) was collected for VWF. Further, the Hb-bound VWF was isolated from above VWF fraction using an Ni-NTA affinity column. The VWF antigen level was measured using a commercial kit. The Hb VWF multimers existed as about 14% of total VWF in patients compared to only about 3.5% in normals ( 𝑛 = 5 ). (b): the ristocetin cofactor VWF activity assay shows that the Hb-bound VWF multimers are 35% more activity than the Hb-free counterparts. (c), The collagen binding assay also shows that the Hb-bound VWF multimers are 33% more adhesive to collagen than the Hb-free multimers.
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(a)
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(b)
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(c)