Surgical procedures in patients with Glanzmann's thrombasthenia

Abstract

Glanzmann's thrombasthenia is a rare platelet function disorder with an autosomal recessive pattern of inheritance. Achieving haemostasis in such patients who undergo surgical procedures always poses a significant challenge. Herein we report six cases of Glanzmann's thrombasthenia, who underwent nine surgeries under the cover of platelet-rich concentrates with or without recombinant activated factor VII . Of these, five were major surgeries such as thyroidectomy, laparotomy, Hartmann's procedure, reversal of Hartmann's procedure and a complete dental extraction. All five procedures were successfully done without any major bleeding. The major cost incurred in these procedures is due to the large number of blood products used and recombinant activated factor VII if used.

Glanzmann’s thrombasthenia with spontaneous upper gastrointestinal bleeding: a case report

“…Increasing numbers of cases of GT have been reported in recent years. 7 Purpura, mucosal bleeding, and menorrhea are the most common clinical features of GT. Most hemorrhagic signs are caused by trauma, surgery, or delivery; idiopathic bleeding is infrequent.…”

Glanzmann Thrombasthenia: Perspectives from Clinical Practice on Accurate Diagnosis and Optimal Treatment Strategies

“…In Type I GT, platelets’ membrane expression of α IIb β 3 integrin is less than 5% of the wild-type quantity. 47 , 48 Type I GT is most common, representing 62–78% of GT cases. 1 , 3 , 4 , 20 Type II GT, in which 5–25% of normal of α IIb β 3 integrin expression is maintained, 4 , 8 , 20 , 47 , 48 represents about 12–16% of the GT population.…”

“… 47 , 48 Type I GT is most common, representing 62–78% of GT cases. 1 , 3 , 4 , 20 Type II GT, in which 5–25% of normal of α IIb β 3 integrin expression is maintained, 4 , 8 , 20 , 47 , 48 represents about 12–16% of the GT population. 1 , 3 , 4 , 20 Type III represents a “variant” GT phenotype in which the α IIb β 3 integrin is present in sufficient quantities at the platelet membrane (ranging from 25% to 100% of reference levels), 8 , 20 , 47 , 48 but is qualitatively dysfunctional, and represents 8–22% of affected patients.…”

“… 1 , 3 , 4 , 20 Type II GT, in which 5–25% of normal of α IIb β 3 integrin expression is maintained, 4 , 8 , 20 , 47 , 48 represents about 12–16% of the GT population. 1 , 3 , 4 , 20 Type III represents a “variant” GT phenotype in which the α IIb β 3 integrin is present in sufficient quantities at the platelet membrane (ranging from 25% to 100% of reference levels), 8 , 20 , 47 , 48 but is qualitatively dysfunctional, and represents 8–22% of affected patients. 1 , 3 , 4 , 20 Mutations conferring a defective α IIb β 3 integrin result in varying clinical severities, but tend to involve ligand binding sites, such as ITGB3 c.719G>A, and inside-out signaling, such as ITGB3 c.2332T>C. 8 , 45 , 49 , 50 Interestingly, gain-of-function GT-like cases have also been described involving compound heterozygous ITGA2B and ITBG3 mutations affecting membrane-adjacent residues resulting in auto-activation of α IIb β 3 , reduced α IIb β 3 expression, and thrombocytopenia.…”

“…Cold compresses are not recommended, as they have been shown to impair haemostasis and coagulation in patients with and without bleeding disorders. 98 , 99 The use of oral antifibrinolytic therapy, 31 , 47 including tranexamic acid (TXA) and aminocaproic acid, can be easily administered in the home setting and may be critical for stopping a bleed in its early stages. Antifibrinolytics should not be administered to patients experiencing gross hematuria, however.…”