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The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia.

ABSTRACT: Standard treatment for Glanzmann thrombasthenia is platelet transfusion. Recombinant activated factor VII has been shown to be successful in patients with Glanzmann thrombasthenia with platelet antibodies or who are refractory to platelet transfusions. The Glanzmann Thrombasthenia Registry prospectively collected worldwide information on the effectiveness and safety of platelet transfusion, recombinant activated factor VII and/or antifibrinolytics for the treatment of bleeds in patients with Glanzmann thrombasthenia. Data relating to 829 non-surgical bleeding episodes were entered into the Glanzmann Thrombasthenia Registry (severe/moderate: 216/613; spontaneous/post-traumatic: 630/199). Recombinant activated factor VII alone was used in 124/829 bleeds, recombinant activated factor VII+antifibrinolytics in 107/829, platelets±antifibrinolytics in 312/829, antifibrinolytics alone in 219/829, and recombinant activated factor VII+platelets±antifibrinolytics in 67/829. The proportion of successful treatments to stop bleeding was 91.0% in cases treated with recombinant activated factor VII only, 82.7% for recombinant activated factor VII+antifibrinolytics, 72.7% for treatment with recombinant activated factor VII+platelets±antifibrinolytics, 78.8% for platelets±antifibrinolytics and 84.7% for antifibrinolytics alone. Treatment failure was documented in 18 bleeding events (2% of the total treatments), the majority of which were in patients receiving treatment with antifibrinolytics; bleeding re-started in 6% of bleeds after initial effective treatment. Thirty-five adverse events were reported, none of which was a thromboembolic event. Among treatments that included recombinant activated factor VII, only one patient reported three possibly drug-related non-serious adverse events (nausea, dyspnea and headache). To conclude, non-surgical bleeds were common and often severe in Glanzmann thrombasthenia; both platelets and recombinant activated factor VII appeared to be effective, and with good safety profiles, for the treatment of non-surgical bleeds. This trial was registered at clinicaltrials.gov identifier: NCT01476423.

SUBMITTER: Di Minno G

PROVIDER: S-EPMC5004418 | biostudies-literature | 2015 Aug

REPOSITORIES: biostudies-literature

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The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia.

Di Minno Giovanni G Zotz Rainer B RB d'Oiron Roseline R Bindslev Niels N Di Minno Matteo Nicola Dario MN Poon Man-Chiu MC

Haematologica 20150522 8

Standard treatment for Glanzmann thrombasthenia is platelet transfusion. Recombinant activated factor VII has been shown to be successful in patients with Glanzmann thrombasthenia with platelet antibodies or who are refractory to platelet transfusions. The Glanzmann Thrombasthenia Registry prospectively collected worldwide information on the effectiveness and safety of platelet transfusion, recombinant activated factor VII and/or antifibrinolytics for the treatment of bleeds in patients with Gla ...[more]

PMID: 26001793

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Project description:BackgroundGlanzmann thrombasthenia is a rare bleeding disorder due to defects in the glycoprotein Ilb/IIIa complex present on the platelet membrane. The most common mode of treatment for this disorder is platelet transfusion. However, scientific evidence does state that repeated transfusions could lead to auto immunization making transfusions ineffective.Aim and objectiveTo describe the use of a novel technique of soft splint with tranexamic acid paste to stop oral bleeding in a patient with Glanzmann thrombasthenia (GT).Case descriptionA 7-year-old female patient with a known history of GT was referred to the pediatric department. The chief complaint of the patient revealed a history of spontaneous bleeding from the oral cavity. The patient required multiple teeth extractions due to severely carious teeth. History revealed that the patient was admitted to the hospital (casualty) three times within 1 month due to a similar complaint leading to hypovolemic shock. Several transfusion procedures were done to stop the spontaneous bleeding. To stop the intraoral bleeding without transfusions, a novel method of using a soft splint with tranexamic acid paste (500 mg tablet crushed and mixed with saline) in the area of spontaneous bleeding was employed with a successful follow-up of 7 months.ConclusionSoft splint with the tranexamic acid paste can serve as an effective method of treatment for patients with spontaneous oral bleeding due to medical conditions like GT.Clinical relevanceThis case report highlights the need for awareness among all the healthcare providers about the importance of regular dental visits. The healthcare providers also need to emphasize the same to all patients with medical conditions to avoid such life-threatening situations.How to cite this articleBhavyaa R, Vignesh KC, Muthu MS, et al. Glanzmann Thrombasthenia: Use of the Soft Splint with Tranexamic Acid Paste to Reduce Spontaneous Oral Bleeding. Int J Clin Pediatr Dent 2021;14(4):580-585.

Dataset Information

The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia.

Publications

The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia.

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