Thromb Haemost 1991; 65(03): 306-311
DOI: 10.1055/s-0038-1648140
Original Article
Schattauer GmbH Stuttgart

Abnormal Platelet Functions in a Patient with Abetalipoproteinemia

Ingrid I Surya
The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Marlene Mommersteeg
The Department of Haematology, University Hospital Utrecht, The Netherlands
,
Gertie Gorter
The Department of Haematology, University Hospital Utrecht, The Netherlands
,
D Willem Erkelens
*  The Internal Medicine, University Hospital Utrecht, The Netherlands
,
Jan-Willem N Akkerman
The Department of Haematology, University Hospital Utrecht, The Netherlands
› Author Affiliations
Further Information

Publication History

Received: 04 July 1990

Accepted after revision 30 October 1990

Publication Date:
02 July 2018 (online)

Summary

Studies with isolated lipoproteins and washed platelets suggest that lipoproteins may affect platelet functions. We investigated platelet-rich plasma (PRP) from a patient with abetalipoproteinemia (ABL), whose plasma lacks apo-B containing lipoproteins (VLDL, LDL and chylomicrons). ABL-PRP aggregated poorly with different agonists and failed to respond to arachidonate. Thromboxane. B2 (T×B2) formation was severely impaired. After gel-filtration most of the aggregation defects persisted in agreement with reduced metabolism of endogenous arachidonate. However, arachidonate-induced aggregation and T×B2 production partially normalized. Normal platelets suspended in ABL plasma showed similar defects in aggregation and T×B2 production but arachidonate-induced aggregation was much lower than expected on the basis of T×B2. We conclude that the abnormal platelet functions in ABL-PRP are caused by (i) an intrinsic platelet abnormality due to reduced arachidonate mobilization and (ii) a property in ABL plasma that inhibits aggregation partially by trapping the arachidonate and partially by an unidentified mechanism. The latter properties may be the result of the abnormal lipid composition of ABl-plasma.