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Thromboelastographic Evaluation of Recombinant Factor VIIa in a Child with Glanzmann's Thrombasthenia

Ismael, F; van der Meer, J; de Wolf, J Th. M.; Kuizenga, K; Aarts, L P. H. J.; Hendriks, H G. D.

Section Editor(s): Shafer, Steven L.

doi: 10.1213/01.ANE.0000227152.05431.67
Letters to the Editor: Letters & Announcements
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Department of Anaesthesiology (Ismael)

Division of Hemostasis, Thrombosis and Rheology (van der Meer)

Department of Hematology (de Wolf)

Department of Anaesthesiology; University Medical Centre Groningen; Groningen, the Netherlands; f.ismael@anest.umcg.nl (Kuizenga, Aarts, Thomas)

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To the Editor:

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive inherited disorder of platelet function (1). Recombinant factor VIIa (rFVIIa; NovoSeven) has been introduced as an alternative to platelet transfusion in patients with GT-associated bleeding (2–5). Conventional laboratory tests of hemostasis, prothrombin time, and activated partial thromboplastin time show normal values in GT patients; they are not influenced by rFVIIa administration. We used thromboelastography (TEG®; Haemoscope, Niles, IL) to monitor the effects of rFVIIa on hemostasis in an 11-yr-old boy with GT referred to our hospital for orchidopexy.

To reduce perioperative bleeding, we administered an IV bolus of 90 μg/kg rFVIIa 10 min before surgery. We recorded TEG® tracings before and after administering rFVIIa (Figure 1). We repeated the dose twice at 2-h intervals after surgery. The patient recovered without excessive bleeding. No adverse events were observed during 6-mo follow-up. Repeated TEG® (Figure 1) at 6 mo showed values comparable to those at baseline, except for a longer reaction time (r): 73.3 versus 49.3 min.

Figure 1.

Figure 1.

In contrast to conventional laboratory test results, TEG® clearly showed a defect in this GT patient's hemostasis. Prolonged r and k values and a decreased α-angle indicated delayed clot formation, and a decreased maximum amplitude (MA) value indicated impaired clot strength. After we administered rFVIIa r, k, and α, values normalized, and the MA improved, though the latter did not normalize. These findings suggest normalized speed of clot formation and improved clot strength.

In GT patients, dosages and number of doses of rFVIIa administration are based on the clinical response, i.e., stopping or preventing bleeding. Clinical titration can result in over- or under-dosage of rFVIIa and conflicting treatment protocols (2–5). Moreover, GT patients can be classified according to the number of platelet glycoprotein IIb/IIIa receptors expressed on their platelet surface. Bleeding tendency in GT patients may vary accordingly. TEG®, which may enable both identification and quantification of the hemostatic defect in GT patients, can be used as a simple test to monitor the effects of corrective treatment.

F. Ismael

Department of Anaesthesiology

J. van der Meer

Division of Hemostasis, Thrombosis and Rheology

J. Th. M. de Wolf

Department of Hematology

K. Kuizenga

L. P. H. J. Aarts

H. G. D. Hendriks

Department of Anaesthesiology

University Medical Centre Groningen

Groningen, the Netherlands

f.ismael@anest.umcg.nl

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REFERENCES

1. George JN, Caen JP, Nurden AT. Glanzmann's thrombasthenia: the spectrum of clinical disease. Blood 1990;75:1383–95.
2. Poon M-C, d'Oiron R. Recombinant activated factor VII (NovoSeven) treatment of platelet-related bleeding disorders. International Registry on Recombinant Factor VIIa and Congenital Platelet Disorders Group. Blood Coagul Fibrinolysis 2000; 11.S:S55–68.
3. Caglar K, Cetinkaya A, Aytac S, et al. Use of recombinant factor VIIa for bleeding in children with Glanzmann thrombasthenia. Pediatric Hematol Oncol 2003;20: 435–8.
4. Yilmaz BT, Alioglu B, Ozyurek E, et al. Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with Glanzmann thrombasthenia. Pediatr Cardiology 2005;26: 843–5.
5. Poon MC, Dóiron R, Von Depka M, et al. Prophylactic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia; result of an international survey. J Throm Haemost 2004;2:1096–103.
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