Atherosclerosis and Unstable Angina in Bernard-Soulier Syndrome

Humphries, John Eric; Yirinec, Bruce A.; Hess, Charles E.

doi:10.1093/ajcp/97.5.652

Cited by 16 publications

(11 citation statements)

References 25 publications

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“…10 The finding is still difficult to extrapolate to man, especially in the face of a report of an acute coronary syndrome in a BSS patient. 5 However, the GPIb␤ Ϫ/Ϫ model should permit further evaluation of the role of this receptor in thrombosis under atherosclerotic conditions, particularly after crossing with APOE-or LDLR-deficient mice. …”

Section: Figure 5 Resistance To Thrombosis Of Gpib␤mentioning

confidence: 99%

“…One report described the occurrence of unstable angina in a Bernard-Soulier patient, suggesting that the absence of the complex does not fully protect against thrombosis. 5 Indications of decreased thrombosis have also come from assays involving perfusion of Bernard-Soulier blood over thrombogenic surfaces such as exteriorized blood vessels or collagen. 6,7 Mice deleted of the GPIb␣ or GPIb␤ subunit have been reported to reproduce the bleeding and platelet morphological defects found in Bernard-Soulier patients, but there is as yet no information concerning their thrombotic tendency.…”

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confidence: 99%

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Decreased Thrombotic Tendency in Mouse Models of the Bernard-Soulier Syndrome

Strassel

Nonne

Eckly

et al. 2007

ATVB

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Objective-The platelet glycoprotein (GP)Ib-V-IX complex is a receptor required for normal hemostasis deficient in the Bernard-Soulier bleeding disorder. To evaluate the consequences of GPIb-V-IX deficiency in thrombosis we generated mouse models of the disease by targeting the GPIb␤ subunit. Methods and Results-Complete deletion (GPIb␤ Ϫ/Ϫ ) or an intracellular truncation (GPIb␤⌬IC Ϫ/Ϫ ) reproduced typical and variant forms of Bernard-Soulier, with absent and partial (20%) expression of the complex on the platelet surface. Both strains exhibited thrombocytopenia and enlarged platelets with abnormal microtubular structures but normal granule composition. They exhibited prolonged tail bleeding times, which were less pronounced in GPIb␤⌬IC Ϫ/Ϫ . Decreased thrombus formation was observed after blood perfusion over a collagen coated surface at high shear. Resistance to vascular occlusion and an abnormal thrombus composition were observed in a model of FeCl 3 -induced lesion of carotid arteries. In a model of laser-induced lesion of mesenteric arterioles, thrombosis was strongly reduced in GPIb␤ Ϫ/Ϫ mice, while a more modest effect was observed in GPIb␤⌬IC Ϫ/Ϫ animals. Finally, the two strains were protected against death in a model of systemic thromboembolism. Conclusions-This

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Section: Figure 5 Resistance To Thrombosis Of Gpib␤mentioning

confidence: 99%

mentioning

confidence: 99%

Decreased Thrombotic Tendency in Mouse Models of the Bernard-Soulier Syndrome

Strassel

Nonne

Eckly

et al. 2007

ATVB

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“…The failure of ATA to prevent thrombotic occlusion may be due to factors other than vWF and GPlb. This hypothesis is supported by the clinical observation that some patients with von Willebrand disease still develop acute myocardial infarction (Humphries et al, 1992). The data suggest that a blockade of the vWF-GPlb pathway cannot necessarily prevent arterial thrombosis.…”

Section: Discussionmentioning

confidence: 74%

A comparative study of the antithrombotic effect of aurintricarboxylic acid on arterial thrombosis in rats and guinea‐pigs

Takiguchi

Shimazawa

Nakashima

1996

British J Pharmacology

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. The antithrombotic effect of aurintricarboxylic acid (ATA) which inhibits binding of von Willebrand factor (vWF) to platelet glycoprotein lb (GPlb) receptor was evaluated in photochemically‐induced thrombosis models in the femoral artery of rats and guinea‐pigs. . ATA at a dose of 10 mg kg−1 significantly prolonged the time required for thrombotic occlusion of the artery in rats. The antithrombotic efficacy was associated with a significant inhibition of platelet retention and ex vivo botrocetin‐induced platelet aggregation. . On the other hand, in guinea‐pigs, ATA at the same dose inhibited the platelet retention and the platelet aggregation, but did not prevent thromboocclusion. . ATA inhibited aggregation of washed platelets from rats or guinea‐pigs in response to botrocetin and thrombin in a dose‐dependent manner (1–30 μm), and to the same extent. . ATA moderately increased activated partial thromboplastin time and bleeding time in both species. . These results indicate that vWF may play a role in the development of occlusive arterial thrombosis in the rat, but not in the guinea‐pig. . The antithrombotic activity of ATA may partly arise from its inhibitory effect on thrombin, in addition to that on the vWF‐GPlb pathway.

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“…However, because of the rarity of BSS, no established protocols for the perioperative management exist, especially for complication-prone cancer patients. We found five reports in the literature describing patients with BSS who underwent surgical procedures under general anesthesia (Table 1) [13][14][15][16][17]. None of the reports referred to a case of oncological surgery.…”

Section: Discussionmentioning

confidence: 99%

Successful perioperative management for a breast cancer patient with Bernard–Soulier syndrome

Okita¹,

Hihara²,

Konishi³

et al. 2010

Breast Cancer

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We herein report the first case of a Bernard-Soulier syndrome (BSS) patient undergoing a surgical procedure for breast cancer. BSS is a rare hereditary thrombocytopathy associated with defects of the platelet glycoprotein complex glycoprotein Ib/V/IX and characterized by large platelets, thrombocytopenia, and severe bleeding symptoms. Because of the rarity of BSS, there are as yet no defined protocols for the perioperative management, which can be very complex and challenging in patients with coagulopathies, in particular BSS. In this case we successfully performed both an interventional examination as well as mastectomy with axillary lymph node dissection, under the preventive and intermittent transfusion of platelets. No intra- or postoperative bleeding complications occurred. Unfortunately, the patient was diagnosed as having metastatic disease involving liver, lungs, and bones 10 months after the surgery. She had received 1st, 2nd, and 3rd line chemotherapy without severe adverse events. However, gastrointestinal bleeding appeared after she was treated with 4th line chemotherapy. Finally, she succumbed 22 months after the breast surgery.

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Atherosclerosis and Unstable Angina in Bernard-Soulier Syndrome

Cited by 16 publications

References 25 publications

Decreased Thrombotic Tendency in Mouse Models of the Bernard-Soulier Syndrome

Decreased Thrombotic Tendency in Mouse Models of the Bernard-Soulier Syndrome

A comparative study of the antithrombotic effect of aurintricarboxylic acid on arterial thrombosis in rats and guinea‐pigs

Successful perioperative management for a breast cancer patient with Bernard–Soulier syndrome

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