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746 Glazmann Thrombasthenia: Single Center Experience
  1. B Kutlubay,
  2. N Ozdemir,
  3. G Tuysuz,
  4. H Apak,
  5. T Celkan
  1. Pediatric Hematology-Oncology Dept., Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey

Abstract

Aim Glazmann thrombasthenia is a rare autosomal recessive disease characterized by defect in platelet aggregation. Here we report the management of children with Glazmann thrombasthenia followed at Cerrahpasa Medical Faculty Pediatric Hematology Department.

Methods Nineteen patients’ (42% girls, 58% boys; median age: 10 months) files were retrospectively reviewed.

Results The median age of the start of bleeding symptoms was 9 months (2 weeks-24 months). All patients presented with easy bruising and mucosal bleeding. Fourteen patients’ parents were consanguineous. In 15 patients, flow cytometry was performed. According to this, 7 had type I, 6 had type II and 2 had type III disease. Nine patients were treated by thrombocyte transfusion, tranexamic acid, recombinant active factor VII and fibrin glue as a single or combined therapy in invasive procedures; none of them had a major bleeding complication.

Conclusion Bleeding control of invasive procedures may be challenging in children with Glazmann thrombasthenia; local treatments, DDAVP, steroid and antifibrinolytics may be used with success.

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