Shortening of Bleeding Time by 1-Deamino-8-Arginine Vasopressin (DDAVP) in the Absence of Platelet von Willebrand Factor in Gray Platelet Syndrome

Pfueller, Sharron L.; Howard, Margaret A.; White, James G.; Menon, C.; Berry, Elizabeth W.

doi:10.1055/s-0038-1646056

Cited by 34 publications

(13 citation statements)

References 25 publications

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“…The vWF:Ag concentration in plasma is still increased after 8 h in contrast to the platelet adhesiveness, which is back to baseline after about 3 h. As the relative proportion of large vWF multimers does not change during the ®rst 8 h after desmopressin, we do not believe that the platelet stimulation can be explained by the release of unusually large vWF multimers. Pfueller et al [21] found that prominent satellite bands appeared in the middle to low molecular weight multimer region of the vWF, but they could not explain the signi®cance of these bands. We made a similar observation in this study, but it is not probable that these bands have any importance for platelet function.…”

Section: Discussionmentioning

confidence: 96%

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Effect kinetics of desmopressin‐induced platelet retention in healthy volunteers treated with aspirin or placebo

et al. 2000

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Desmopressin is often used for haemostatic treatment in platelet dysfunction, but the effect kinetics of platelet responses and the mechanism of action are poorly known. This study aimed to determine the kinetics of platelet function responses induced by desmopressin in healthy volunteers treated with aspirin or placebo. Another aim was to correlate platelet responses to changes of von Willebrand factor (vWF) in plasma. We measured platelet function with a glass bead retention test, Ivy bleeding time, vWF:Ag and multimeric structure in plasma. Median baseline platelet retention was 12% (normal reference range 16-27%) during aspirin treatment and 18% during placebo. Median peak platelet retention after desmopressin was 33% during aspirin treatment and 34% during placebo. After about 3 h platelet function had returned to baseline. A second desmopressin dose after 3 h stimulated platelet retention to a similar extent as the first dose. There was no correlation between platelet responses and quantitative or qualitative changes of vWF in plasma. Platelet count did not change significantly. Thus, desmopressin's effect on platelet function lasts for about 3 h, but may be prolonged by a second dose immediately thereafter. These findings may have important clinical implications for patients with aspirin-induced platelet dysfunction undergoing surgery.

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Section: Discussionmentioning

confidence: 96%

“…Pfueller et al . [ 21] found that prominent satellite bands appeared in the middle to low molecular weight multimer region of the vWF, but they could not explain the significance of these bands. We made a similar observation in this study, but it is not probable that these bands have any importance for platelet function.…”

Section: Discussionmentioning

confidence: 99%

Effect kinetics of desmopressin‐induced platelet retention in healthy volunteers treated with aspirin or placebo

et al. 2000

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“…Taking into account the data from these case series and from other numerous smaller series and case reports [6,21,23,36,45,[51][52][53][54][55][56][57][58][59][60][61][62], inherited disorders of platelet function that usually respond with a shortening of BT after DDAVP administration include defects of secretion (in particular other than d-storage pool deficiency) and of signal transduction and the May-Hegglin anomaly; an effect has been variably reported in Bernard-Soulier syndrome and rarely shown in GlanzmannÕs thrombasthenia and cyclo-oxygenase deficiency ( Table 3). The response may be broadly predicted by the BT at baseline, perhaps indicating that desmopressin is less useful in more severe platelet function abnormalities showing longer BT.…”

Section: Clinical Evidence Of Ddavp In Inherited Disorders Of Platelementioning

confidence: 99%

Desmopressin in inherited disorders of platelet function

Coppola

Minno

2007

Haemophilia

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Following the first clinical use in haemophilia and von Willebrand disease in 1977, the synthetic analogue of vasopressin 1-deamino-8-D-arginine vasopressin (DDAVP, desmopressin) was successfully employed for the management of a series of bleeding disorders, including congenital and acquired defects of platelet function. In this setting, few haemostatic approaches are available and, in particular for severe bleeding and major invasive procedures, the transfusion of platelet concentrates is the first-choice treatment. Therefore, DDAVP was (and remains) an attractive therapeutic alternative, being well tolerated, cost-saving, administrable at home (by the intranasal or subcutaneous concentrated formulations) and, in particular, enabling the avoidance of blood product exposition and the related risks (allergic reactions, transfusion transmitted infections). Despite three decades of clinical use, cellular mechanisms of haemostatic effects of DDAVP in platelet defects remain poorly known and the excellent results reported in some case series have not been strengthened by rigorous clinical trials, hampered by the rarity and the heterogeneity of these disorders. However, clinical experience more than evidence-based medicine reserved an established place to DDAVP in the management of inherited platelet disorders. This review will focus the available clinical data and the open issues of DDAVP in this setting.

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“…More recently, one family with an autosomal-dominant form of GPS associated with a dominant-negative GFI1B mutation was described [9]. Different abnormalities of platelet hemostatic functiondefective response to thrombin [10][11][12][13][14] and collagen [10][11][12][14][15][16][17][18], variable response to ADP and ristocetin [10-12, 14, 17, 18] and normal response to arachidonic acid [10,11,14] -have been described so far in GPS patients. In one case, a specific defect of GPVI associated with abnormal response to collagen and convulxin was described [17].…”

Section: Introductionmentioning

confidence: 98%

Megakaryocytic emperipolesis and platelet function abnormalities in five patients with gray platelet syndrome

et al. 2015

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The gray platelet syndrome (GPS) is a rare congenital platelet disorder characterized by mild to moderate bleeding diathesis, macrothrombocytopenia and lack of azurophilic α-granules in platelets. Some platelet and megakaryocyte (MK) abnormalities have been described, but confirmative studies of the defects in larger patient cohorts have not been undertaken. We studied platelet function and bone marrow (BM) features in five GPS patients with NBEAL2 autosomal recessive mutations from four unrelated families. In 3/3 patients, we observed a defect in platelet responses to protease-activated receptor (PAR)1-activating peptide as the most consistent finding, either isolated or combined to defective responses to other agonists. A reduction of PAR1 receptors with normal expression of major glycoproteins on the platelet surface was also found. Thrombin-induced fibrinogen binding to platelets was severely impaired in 2/2 patients. In 4/4 patients, the BM biopsy showed fibrosis (grade 2-3) and extensive emperipolesis, with many (36-65%) MKs containing 2-4 leukocytes engulfed within the cytoplasm. Reduced immunolabeling for platelet factor 4 together with normal immunolabeling for CD63 in MKs of two patients demonstrated that GPS MKs display an alpha granule-specific defect. Increased immunolabeling for P-selectin and decreased immunolabeling for PAR1, PAR4 and c-MPL were also observed in MKs of two patients. Marked emperipolesis, specific defect of MK alpha-granule content and defect of PAR1-mediated platelet responses are present in all GPS patients that we could study in detail. These results help to further characterize the disease.

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Shortening of Bleeding Time by 1-Deamino-8-Arginine Vasopressin (DDAVP) in the Absence of Platelet von Willebrand Factor in Gray Platelet Syndrome

Cited by 34 publications

References 25 publications

Effect kinetics of desmopressin‐induced platelet retention in healthy volunteers treated with aspirin or placebo

Effect kinetics of desmopressin‐induced platelet retention in healthy volunteers treated with aspirin or placebo

Desmopressin in inherited disorders of platelet function

Megakaryocytic emperipolesis and platelet function abnormalities in five patients with gray platelet syndrome

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