J Neurocrit Care.  2017 Dec;10(2):126-128. 10.18700/jnc.170025.

Glanzmann Thrombasthenia in a Korean Patient: A Postoperative Intracranial Hemorrhagic Diasthesis in a Patient with a Rupture of a Cerebral Aneurysm

Affiliations
  • 1Department of Neurosurgery, Ajou University School of Medicine, Suwon, Korea. nsyclim@gmail.com

Abstract

BACKGROUND
Glanzmann thrombasthenia (GT) is a rare disease with severe insufficiency of platelet function. There are no reports of GT associated with a cerebral hemorrhage.
CASE REPORT
A 45-year-old woman had a stuporous mentality. Her computed tomographic (CT) angiography revealed a rupture of the posterior cerebral artery aneurysm. Hunt-Hess grade was 4 points and Fisher grade was 3 points. A clipping of the aneurysm was performed and proceeded to external ventricular drainage for hydrocephalus after 6 days. But, a new hemorrhage was detected several times via a follow-up brain CT. Various blood tests were conducted to evaluate the bleeding tendency, and the GT was diagnosed with PFA-200. The ventriculoperitoneal shunt was performed after the platelets transfusion, and she was discharged without other complications.
CONCLUSION
With nonspecific and recurrent intracranial hemorrhage, a GT should be included in the differential diagnosis, which is important in controlling bleeding during the surgery.

Keyword

Trombastniahe; Postoperative hemorrhages; Aneurysm, Ruptured

MeSH Terms

Aneurysm
Aneurysm, Ruptured
Angiography
Blood Platelets
Brain
Cerebral Hemorrhage
Diagnosis, Differential
Drainage
Female
Follow-Up Studies
Hematologic Tests
Hemorrhage
Humans
Hydrocephalus
Intracranial Aneurysm*
Intracranial Hemorrhages
Middle Aged
Postoperative Hemorrhage
Rare Diseases
Rupture*
Stupor
Thrombasthenia*
Ventriculoperitoneal Shunt

Figure

  • Figure 1. Brain computed tomography finding shows the status of the clipping of an aneurysm and inserting an intraoperative external ventricular drainage catheter (A, B). A new hemorrhage was observed at the removal site of the existing catheter in the right side and at the insertion site of the new catheter in the left side (C). The expansion of the hemorrhage around catheter in the left side was detected (D).


Reference

1. Toygar HU, Guzeldemir E. Excessive gingival bleeding in two patients with Glanzmann thrombasthenia. J Periodontol. 2007; 78:1154–8.
Article
2. Nurden AT, Pillois X, Wilcox DA. Glanzmann thrombasthenia: state of the art and future directions. Semin Thromb Hemost. 2013; 39:642–55.
Article
3. Solh T, Botsford A, Solh M. Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015; 6:219–27.
4. Franchini M, Lippi G. NovoSeven (recombinant factor VIIa) for the treatment of bleeding episodes and perioperative management in patients with Glanzmann's thrombasthenia. Expert Rev Hematol. 2014; 7:733–40.
Article
5. George JN, Caen JP, Nurden AT. Glanzmann's thrombasthenia: the spectrum of clinical disease. Blood. 1990; 75:1383–95.
Article
6. Toogeh G, Sharifian R, Lak M, Safaee R, Artoni A, Peyvandi F. Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran. Am J Hematol. 2004; 77:198–9.
Article
7. Tabibian S, Motlagh H, Naderi M, Dorgalaleh A. Intracranial hemorrhage in congenital bleeding disorders. Blood Coagul Fibrinolysis. 2017; [Epub ahead of print].
Article
8. Yougbaré I, Lang S, Yang H, Chen P, Zhao X, Tai WS, et al. Maternal anti-platelet β3 integrins impair angiogenesis and cause intracranial hemorrhage. J Clin Invest. 2015; 125:1545–56.
Article
9. Iqbal I, Farhan S, Ahmed N. Glanzmann thrombasthenia: a clinicopathological profile. J Coll Physicians Surg Pak. 2016; 26:647–50.
10. Segna E, Artoni A, Sacco R, Giannì AB. Oral surgery in patients with Glanzmann thrombasthenia: a case series. J Oral Maxillofac Surg. 2017; 75:256–9.
Article
11. Nurden AT, Fiore M, Nurden P, Pillois X. Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models. Blood. 2011; 118:5996–6005.
Article
12. Fiore M, Nurden AT, Nurden P, Seligsohn U. Clinical utility gene card for: Glanzmann thrombasthenia. Eur J Hum Genet. 2012; 20.
Article
Full Text Links
  • JNC
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr