Possible Thrombosthenin Defect in Glanzmann’s Thrombasthenia

Possible thrombosthenin defect in Glanzmann's thrombasthenia.

Platelet thrombosthenin: subcellular localization and function.

Thrombosthenin, an immunologically distinct contractile protein was isolated in relatively pure form from human platelets. The protein, which was of high molecular weight appeared to be composed of multiple polypeptide subunits, probably polymeric in nature.Thrombosthenin had magnesium-dependent ATPase activisty, releasing an average of 3 mug phosphorus per mg protein in 30 min. After the addit...

Glanzmann thrombasthenia

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of alphaIIb beta3 integrin. This receptor mediates the binding of adhesive proteins that attach aggregating platelets and ensure thrombus formation at si...

Renal embolisation in Glanzmann's thrombasthenia.

The patient, a 58-year-old woman, had suffered from excessive bleeding since early childhood. The coagulation studies were diagnostic of Glanzmann's thrombasthenia with diminished platelet aggregation in response to ristocetin (table). She was admitted to hospital because of intractable haematuria for which no cause could be established. There were no signs of urinary tract infection or stones....

Glanzmann thrombasthenia in a neonate.

Glanzmann thrombasthenia is a qualitative platelet function disorder manifested by skin bleeds, epistaxis, gingival bleeding, gastrointestinal hemorrhage, hematuria, hemarthrosis, intracranial hemorrhage and visceral hematomas. We report a six day old newborn presenting with hematuria following suprapubic aspiration, who was diagnosed as Glanzmann thrombasthenia. We believe it to be the younges...