Cases reported "Blood Platelet Disorders"

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1/4. Glanzmann's thrombasthenia. Report of two oral surgical cases using a new microfibrillar collagen preparation and EACA for hemostasis.

    Glanzmann's thromboasthenia is a rare congenital platelet disorder characterized by a prolonged bleeding time, a qualitative platelet defect, and severe hemorrhagic episodes. patients with this disorder have been managed by administration of blood and blood components (most recently, platelet-rich plasma and platelet concentrates) to control hemorrhage resulting from trauma or surgical procedures. The two case reports presented here illustrate the use of a local hemostatic agent (microfibrillar bovine collagen, Avitene) and a systemic fibrinolytic inhibitor (epsilon aminocaproic acid, Amicar) to control postoperative hemorrhage secondary to elective extraction of teeth. The clinical results demonstrate excellent postoperative hemostasis and support recent in vitro observation of platelet adherence to the collagen preparation. This provides an alternate therapeutic modality in the management of patients with Glanzmann's disease and possibly other disorders of platelet function.
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2/4. Multiple dental extractions in a child with Glanzmann's thrombasthenia: report of case.

    patients with Glanzmann's thrombasthenia have a tendency to hemorrhage. This report shows evidence, however, that extractions in children with the disease can be accomplished without supplemental therapy.
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3/4. The management of dental extractions in cases of thrombasthenia complicated by the development of isoantibodies to donor platelets.

    Two cases of thrombasthenia, a rare hereditary disorder of platelet function, are presented. The oral surgical and dental management of these cases is discussed in the light of the development of isoantibodies to transfused platelets in one of the cases and in another case encountered. The problems of obtaining donor platelets matched for platelet and HL-A antigens are discussed. Emphasis is placed on the use of local hemostatic measures and antifibrinolytic agents in the management of hemorrhage in this disorder and the avoidance of platelet transfusions as far as possible. Conservative dentistry and early preventive dental advice are considered desirable in order to avoid extractions.
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4/4. Deficiency of p-selectin in a patient with grey platelet syndrome.

    Patient B.G. is a 29-yr-old female with a lifelong bleeding disorder characterized clinically by a highly increased bleeding time, menorrhagias, long-lasting bleeding after cuts and tooth extractions and large post-traumatic haematomas. Her coagulation tests were within normal range, platelet count was 140,000-160,000 per microliters, but platelet function was impaired as demonstrated by the absence of collagen-induced aggregation, although no abnormalities were detected in aggregation response to ADP and ristocetin. Morphologically her platelets were characterized by gigantic size-average profile area was about 2.5 times higher than that of control donors, and severe deficiency of alpha-granules-only 16% of their number in control donors. These features taken together indicated the diagnosis of grey platelet syndrome. As has been shown by quantitative immunoblotting, patient's platelets contained small amounts of alpha-granule membrane protein p-selectin-about 15% of that in control donors. The content of plasma membrane glycoproteins IIb-IIIa and Ib was not reduced, suggesting the specific deficiency of alpha-granule membrane protein. Thus, B.G. is the second patient described in the literature (see also Lages et al, J Clin Invest 1991: 87: 919-929) with combined deficiency of alpha-granules and p-selectin.
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