Table. 2.

Table. 2.

Agents for the treatment of bleeding in patients with inherited platelet function disorders

Agent Indication and dose Caution
Tranexamic acid PO: 15-25 mg/kg q 8 hours for menorrhagia and mild mucosal bleeding, including epistaxis [2,30] Obstructive uropathy in urinary tract bleeding, hematoma in pleural space bleeding
IV: 10-15 mg/kg q 8 hours for serious bleeding up to q 6 hours in selected cases [2,30]
Mouth wash: 10 mL of a 5% solution 4-6 times a day for local mouth bleeding [2,84]
Aminocaproic acid PO: 60-90 mg/kg q 6-8 hours [84] Shorter half-life, less potency, more toxicity than tranexamic acid [89]
IV: 100 mg/kg over 15 min, followed by 10 mg/kg/h or 5 g bolus 4 h [30]
Desmopressin IV: 0.3 mg/kg in 20-50 mL of saline over 30 min, 1 h before procedure [2,4,30,53,87] Fluid retention, hyponatremia-induced seizure, caution with the use in elderly with cardiovascular disease and children younger than 2 years
0.2 mg/kg with tranexamic acid 10 mg/kg [68]
Not exceeding a total dose of 20 mg [84]
SC: 0.3 mg/kg [2,4]
Nasal spray: 300 mg for an adult, 150 mg for a child with a weight under <40-50 kg [2,4,30]
rFVIIa IV: ≥90 mg (4.5 kIU)/kg/dose, q 2-3 h, ≥3 doses or until hemostasis for GT [88] Thromboembolic complications (rare)

PO, per oral; IV, intravenous; SC, subcutaneous; rFVIIa, recombinant activated factor VII; GT, Glanzmann thrombasthenia.

Clin Pediatr Hematol Oncol 2020;27:1-13
© 2020 Clin Pediatr Hematol Oncol