[MIM 273 800]

Unknown prevalence. Autosomal recessive transmission. Hemorrhagic thrombopathic disorder characterized by an absence of platelet aggregation due to an quantitative or qualitative anomaly of a membrane receptor (glycoprotein: integrin aIIbβ3) on the platelet surface. Several of the genes (ITGA2B on 17q21.31 and ITGB3 on 17q21.32) involved in the synthesis of the  glycoprotein IIb/IIIa complex (or fibrinogen receptor) can be affected. This anomaly causes bruising even after minor trauma, frequent mucosal bleeding (gums, nose) but rarely spontaneous bleeding except during menstruation, after trauma or after surgery (or childbirth). 

Depending on the severity of the platelet deficit and depending on the extent of the deficiency in platelet

glycoprotein IIb/IIIa, 3 types are described:

-         type I (severe): level < 5 % of normal; low or absent binding to fibrinogen and absence of clot retraction.

-         type II (less severe): level between 5 % and 20 %; binding to fibrinogen  is present and clot retraction normal or partial.

-         type III (not severe): level > 50 %, no significant abnormality of platelet aggregation; binding to fibrinogen and retraction of the clot are variable.

Some rare acquired cases exist due to the presence of autoantibodies directed against the membranar aIIbβ3 glycoproein.

Diagnosis: according to the type, hemorrhage when the umbilical cord falls, prolonged bleeding, profuse menstrual bleeding

Anesthetic implications: 

Contact a specialist in hemostasis. Contraindication to perimedullary anesthesia and retrobulbar blocks. Avoid intramuscular injections and rectal or nasopharyngeal temperature measurement. Insert central venous catheters under ultrasound guidance, and provide prolonged local compression when removing them.

In case of bleeding: local compression, tranexamic acid (10 mg/kg every 8h. IV or p os).

In the event of severe bleeding: administer platelets 1 hour before surgery, then every 12 to 24 hours. Paradoxically, the number of units to be transfused to correct hemostasis is higher than in case of thrombocytopenia, as the donor platelets mix with the defective recipient platelets. 

The thromboelastogram is useful for assessing platelet function.

If antiplatelet antibodies are present  (against the complex GP IIb-IIIa or antigen of the HLA system) or if blood platelets are not available:  administration of at least 3 doses of recombinant activated factor VII (Novoseven®) at a dose of 90 µg/kg every 2 h.

Do not administer any drugs affecting platelet function: aspirin,NSAIDs. In case of pregnancy in a mother carrying anti GP IIb-IIIa antibodies following platelet transfusions, transient thrombocytopenia can appear in the fetus and the newborn.

References: 

-         Monte S, Lyons G.
Peripartum management of a patient with Glanzmann’s thrombasthenia using Thrombelastograph.
Br J Anaesth 2002;88 :734-8.

-        Uzunlar HI, Eroglu A, Senel AC, Bostan H, Erciyes N. 
A patient with Glanzmann’s thrombasthenia for emergent abdominal surgery. 
Anesth Analg 2004; 99: 1258-60.

-        Wertz D, Boveroux P, Peters P, Lenelle J, Franssen C. 
Surgical resection of a sphenoid wing meningioma in a patient with Glanzmann thrombasthenia. 
Acta Anaesth Belg 2011; 62: 83-6.

-        Monrigal C, Beurrier P, Mercier FJ, Boyer-Neumann C, Gillard P.
Thrombasthénie de Glanzmann et grossesse: rapport d’un cas et revue de la litérature.
Ann Fr Anesth Réanim 2003 ; 22 : 826-30.

-        Léticée N, Kaplan C, Lémery D.
Pregnancy in mother with Glanzmann’s thrombasthenia and autoantibody against GPIIb-IIIa : is there a fœtal risk ?
Eur J Obstetr Gynecol Reprod Biol 2005; 121: 139-42

-        Yilmaz BT, Alioglu B, Ozyurek E, Akay HT, Mercan S, Ozbek N et al.
Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with Glanzmann thrombasthenia.
Pediatr Cardiol 2005; 26 : 843-5.

-        Park JB, Shin YS, Kim SH.
Anesthetic experience for orthopedic surgery on a patient with Glanzmann’s thrombasthenia refractory to platelet transfusion: a case report.
Korean J Anesthesiol 2009; 57: 507-10.

-        Fernandez-Castellano G, Mayorga-Buiza MJ, Gallego-Slano J, Vazquez-Rubio R, Rivero-Garvia M.
Acute epidural hematoma following acute subdural hematoma evacuation in a child with Glanzmann thrombasthenia.
J Neurosurg Anesthesiol 2016; 28: 431-3.

-        Fiore M, Giraudet J-S, Alessi M-C, Falaise C, Desprez D et al.
Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders.
Orphanet Journal of Rare Diseases 2023; 18:171 doi.org/10.1186/s13023-023-02787-2

Updated: July 2023