Abnormalities of platelet aggregation in sickle cell anemia presence of a plasma factor inhibiting aggregation by ristocetin

Sarji, Kay E.; Eurenius, Karl; Fullwood, Catherine; Schraibman, H. B.; Colwell, John A.

doi:10.1016/0049-3848(79)90238-x

Cited by 16 publications

(19 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This disagrees with previous observations on the activation of the coagu lation system in SCD patients [1][2][3][4][5][6][7]. Furthermore, we confirmed the well documented abnormal aggrega tion response to ristocetin in the steady state and dur ing crisis [11,13]. Responses to ADP, collagen and adrenaline were normal, although both normal [8,9] as well as diminished [10,11] aggregation responses to the same agents were reported in the steady state and during crisis [10,11], The inhibited aggregation re sponses to ristocetin in SCD have been ascribed to the existence of a ristocetin-inhibiting agent in the plasma of patients with SCD.…”

Section: Discussioncontrasting

confidence: 56%

“…Responses to ADP, collagen and adrenaline were normal, although both normal [8,9] as well as diminished [10,11] aggregation responses to the same agents were reported in the steady state and during crisis [10,11], The inhibited aggregation re sponses to ristocetin in SCD have been ascribed to the existence of a ristocetin-inhibiting agent in the plasma of patients with SCD. This conclusion was obtained from recombination experiments [11,13] in which normal platelets suspended in SCD plasma failed to aggregate in response to low-dose ristocetin (1.09 and 1.12 mg/ml), while SCD platelets aggregated nor mally to ristocetin when suspended in normal plasma. The presence of HbS does not interfere with the aggregation of platelets in response to ristocetin [22,23].…”

Section: Discussionmentioning

confidence: 97%

“…Normal [8,9] as well as decreased [10,11] aggregation were reported in the steady state, while during crisis, aggregation was found to be diminished [11,12]. There is, however, general agreement that poor ristocetin-induced aggre gation is a feature of both the steady state as well as of crisis [11,13]. Recently, various aspects of the SCD in Saudi Arabia have been reported [14][15][16][17].…”

Section: Introductionmentioning

confidence: 96%

See 2 more Smart Citations

Coagulation Changes in Sickle Cell Disease in Early Childhood

Babiker¹,

Ashong²,

Bahakim³

et al. 1987

Acta Haematol

View full text Add to dashboard Cite

Measurements of the coagulation system were carried out in children with sickle cell disease (SCD) in both steady state and on the 1st day of painful crisis and were compared to age- and sex-matched healthy controls. No significant differences were found in prothrombin time, partial thromboplastin time, thrombin time, reptilase time, plasma fibrinogen, antithrombin III, factor VIIL·C, ristocetin-cofactor (Ri-Cof) and platelet aggregation responses to ADP, collagen and adrenaline. Abnormal aggregation responses to ristocetin were noted in all patients with SCD when compared to controls. Daily measurements during the first 4 days of painful crisis showed significant elevation of fibrinogen and Ri-Cof and enhancement of aggregation to ADP and adrenaline by the 3rd day of crisis. It was concluded that the changes noted, rather than being primarily responsible for the onset of crisis, can only be secondary changes arising from the aetiological factors of crisis, i.e. stasis and acute-phase proteins.

show abstract

Section: Discussioncontrasting

confidence: 56%

Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 96%

See 1 more Smart Citation

Coagulation Changes in Sickle Cell Disease in Early Childhood

Babiker¹,

Ashong²,

Bahakim³

et al. 1987

Acta Haematol

View full text Add to dashboard Cite

show abstract

“…Leichtman & Brewer (1977) described absent RIPA in nine of 10 patients with homozygous sickle cell anaemia, three of three subjects with haemoglobin SC disease, and even two of three individuals with sickle trait. A plasma inhibitor against RIPA was demonstrated in many of these individuals (Leichtman & Brewer, 1977;Sarji et a!, 1979). Ristocetin concentrations of 1.09 or 1-12 mg/ml were used in these studies.…”

Section: G R Buchanan C a Holtkamp And E N Levymentioning

confidence: 99%

Racial Differences in Ristocetin‐induced Platelet Aggregation

1981

View full text Add to dashboard Cite

Several investigators have reported defective ristocetin-induced platelet aggregation (RIPA) in individuals whose red blood cells contain sickle haemoglobin, but the race of control subjects in these studies was not stated. Therefore, maximal amplitude of RIPA was examined in 75 normal whites and blacks, of 16 of whom had sickle trait defined by haemoglobin electrophoresis and sickle prep. Final ristocetin concentrations in platelet rich plasma were 1.1, 1.2 and 1.5 mg/ml. Mean aggregation at 1.1 mg/ml was significantly less in blacks (mean 31%) than in whites (mean 72%) (P less than 0.001). 60% of blacks but only 11% of whites had less than 50% RIPA at 1.1 mg/ml. RIPA was entirely absent in 19% of blacks. Differences in RIPA between black and white subjects were also present at ristocetin concentrations of 1.2 and 1.5 mg/ml but were less striking. RIPA in 25 children with homozygous sickle cell anaemia was similar to that in the normal AA and AS blacks. Differences in RIPA could not be explained by age, sex, presence of sickle haemoglobin, or medications. Addition of normal plasma or platelets did not correct reduced RIPA in seven blacks, and their plasma inhibited normal RIPA responses. Reduced platelet aggregation to low concentrations of ristocetin is a normal finding in many blacks, is not related to the presence of sickle haemoglobin, and appears to be due to a plasma inhibitor against RIPA.

show abstract

“…In hemolytic anemias and especially in sickle cell anemias, where large amounts of ADP are released in the bloodstream, plate let counts are found to be either within the normal limits or increased [9,10], which indicates that intravascular aggregation is not taking place. The in vitro inhibitory effects of 2,3-DPG, 3-PG.…”

Section: Discussionmentioning

confidence: 99%

Structure-Function Relationship of 3-Phosphoglycerate Analogues with Platelet Aggregation and Thromboxane A<sub>2</sub> Formation

Iatridis¹,

Hadd²,

Kotrotsou³

et al. 1987

Pathophysiol Haemos Thromb

View full text Add to dashboard Cite

We have studied the effects of 2,3-diphosphoglycerate (2,3-DPG), 3-phospho-glycerate (3-PG), 3-phosphoglyceraldehyde (3-PGA), 2-phosphoglycerate (2-PG) and β-glycerol phosphate (β-GP) on platelet aggregation and on thromboxane B₂ (TXB₂) formation. The results show that 2,3-DPG, 3-PG, and 3-PGA inhibited platelet aggregation and TXB₂ formation induced by norepinephrine, ADP, epinephrine, and collagen; but they also induced platelet aggregation and TXB₂ formation in the presence of subthreshold concentrations of Na arachidonate. 2-PG and β-GP were inactive. The results also show that there is a structure-function relationship between 2,3-DPG, 3-PG, and 3-PGA with platelet aggregation phenomena and prostaglandin synthesis.

show abstract

Abnormalities of platelet aggregation in sickle cell anemia presence of a plasma factor inhibiting aggregation by ristocetin

Cited by 16 publications

References 13 publications

Coagulation Changes in Sickle Cell Disease in Early Childhood

Coagulation Changes in Sickle Cell Disease in Early Childhood

Racial Differences in Ristocetin‐induced Platelet Aggregation

Structure-Function Relationship of 3-Phosphoglycerate Analogues with Platelet Aggregation and Thromboxane A<sub>2</sub> Formation

Contact Info

Product

Resources

About