Severe Intestinal Bleeding in a Woman with Glanzmann Thrombasthenia
  • Raquel Mesquita
    Serviço de Medicina 2.1, Hospital dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal
  • Inês Santos
    Serviço de Medicina 2.1, Hospital dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal
  • Helena Monteiro
    Serviço de Medicina 2.1, Hospital dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal

Abstract

Glanzmann thrombasthenia (GT) is a rare inherited disorder associated with abnormal platelet function. Non-surgical bleeding is common and can be life-threatening. A 70-year-old woman with GT presented with a 3 week history of melena and asthenia. She had hemoglobin of 3.8 g/dL. Both upper and lower endoscopies were inconclusive. Video capsule endoscopy showed multiple angioectasias and erosions in the distal ileum. Following several days of recurring intestinal hemorrhage, only treatment with recombinant activated factor VII proved to be effective, enabling patient stabilization with no further bleeding. Control and prevention of bleeding among these patients is imperative and remains challenging.

VIEW THE ENTIRE ARTICLE

References

  • Poon MC, d’Oiron R, Zotz RB, Bindslev N, Di Minno MN, Di Minno G, et al. The international, prospective Glanzmann Thrombasthenia Registry: treatment and outcomes in surgical intervention. Haematologica 2015;100:1038–44.

  • Di Minno G, Zotz RB, d’Oiron R, Bindslev N, Di Minno MN, Poon MC. The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes in non-surgical bleeding episodes in patients with Glanzmann thrombasthenia. Haematologica 2015;100:1031–37.

  • Rajpurkar M, Chitlur M, Recht M, Cooper DL. Use of recombinant activated factor VII in patients with Glanzmann’s thrombasthenia: a review of the literature. Haemophilia 2014;20:464–71.

  • Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med 2015;6:219–27.

  • Chitlur M, Rajpurkar M, Recht M, et al. Recognition and management of platelet-refractory bleeding in patients with Glanzmann’s thrombasthenia and other severe platelet function disorders. Int J Gen Med 2017;10:95–99.
  • Views: 1054
    HTML downloads: 156
    PDF downloads: 496


    Published: 2017-12-18
    Issue: Vol. 5 No. 2 (view)


    How to cite:
    1.
    Mesquita R, Santos I, Monteiro H. Severe Intestinal Bleeding in a Woman with Glanzmann Thrombasthenia. EJCRIM 2017;5 doi:10.12890/2017_000796.