Abnormal platelet functions in a patient with abetalipoproteinemia.
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 (TxB2) 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 TxB2 production partially normalized. Normal platelets suspended in ABL-plasma showed similar defects in aggregation and TxB2 production but arachidonate-induced aggregation was much lower than expected on the basis of TxB2. 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.
Surya
I I
II
Department of Haematology, University Hospital Utrecht, The Netherlands.
Mommersteeg
M
M
Gorter
G
G
Erkelens
D W
DW
Akkerman
J W
JW
D000012:Abetalipoproteinemia / Q000097:blood*; D000255:Adenosine Triphosphate / Q000378:metabolism; D000328:Adult; D016718:Arachidonic Acid; D001095:Arachidonic Acids / Q000097:blood; D001792:Blood Platelets / Q000378:metabolism / Q000502:physiology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D010974:Platelet Aggregation / Q000502:physiology
D001095:Arachidonic Acids; D016718:Arachidonic Acid; D000255:Adenosine Triphosphate