Congenital Disorders of Platelet Function

Podda, Gian Marco; Pugliano, Mariateresa; Cattaneo, M.

doi:10.17925/eoh.2008.02.1.43

Cited by 2 publications

(2 citation statements)

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“…Depending on the hematological examination and level of platelets surface glycoproteins, 20 patients were diagnosed with BSS and 23 with GT. Variable thrombocytopenia, large platelets and decreased GPIb/ IX level were seen in BSS patients, while the level of GPIIb/IIIa was decreased in GT cases, the basic characteristics of patients are shown in tables (2).…”

Section: Resultsmentioning

confidence: 99%

“…Inherited platelet function disorders (IPFDs) are a heterogeneous group of bleeding disorders caused by defects of soluble agonist receptors, adhesive protein receptors, platelet granules and membrane phospholipids [1] . Inherited platelet disorders may change the number of circulating platelets as well as their function, cause mucocutaneous bleeding signs with varying intensity, generally bruise easily, epistaxis, ecchymosis and gingival bleeding [2] . Among IPFDs Glanzmann thrombasthenia (GT) and Bernard Soulier syndrome (BSS) are exceedingly rare, but their intensity and biological features make them especially important [3] .…”

Section: Introductionmentioning

confidence: 99%

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Diagnosis of Bernard Soulier Syndrome and Glanzmann’s Thrombasthenia in Iraqi Patients

Lina Mussa Kadhim,

Muthana Ibrahim Malak,

Safa A Faraj

2021

Indian Journal of Forensic Medicine & Toxicology

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Background: Bernard-Soulier syndrome (BSS) and Glanzmann Thrombasthenia (GT) are rare inherited platelet function disorders, defined by a permanent history of mucocutaneous bleeding. The objective of this project was to identify biological and clinical characteristics of BSS and GT patients. Methodology: This study included 52 patients with bleeding disorders, the clinical, haematological and demographic features of patients were determined and the level of GpIIb/IIIa and GPIb/IX was assessed. Results: From a total of 52 patient, 20 were diagnosis with BSS (9 females and 11 males), their age range from 6 to 42 year and 23 diagnoses with GT (10 females and 13 males), their age range from 4 to 50 year. Epistaxis, easy bruising, menorrhagia and ecchymosis were the most frequent symptoms. Prolonged bleeding time (BT), normal PT and PTT were seen in all cases. Variable thrombocytopenia, large platelets with decreased GPIb/IX level were seen in BSS patients, while the level of GPIIb/IIIa was decreased in GT cases. Conclusion: Since BSS and GT are infrequent disorders diagnosis may be postponed, both diseases are combined with crucial bleeding tendency, therefore earlier detection would have been important for patients management.

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Section: Resultsmentioning

confidence: 99%