Dosering

Informasjon på denne siden er på engelsk. 

ADZYNMA is an enzyme replacement therapy (ERT) indicated for the treatment of ADAMTS13 deficiency in children and adult patients with congenital thrombotic thrombocytopenic purpura (cTTP). ADZYNMA can be used for all age groups. [1]

✓ADZYNMA is intended for intravenous use after reconstitution only. ADZYNMA 500 IU and ADZYNMA 1500 IU powder and solvent for solution for injection should be administered at a rate of 2 to 4 mL per minute. [1]

✓ 1 Recommended prophylactic dose is 40 IU/kg of body weight once every other week. The prophylaxis dosing frequency may be adjusted to 40 IU/kg of body weight once weekly based on clinical response. [1]

✓ 1 For on-demand enzyme replacement therapy: In case of acute thrombotic thrombocytopenic purpura (TTP) episode, the recommended dose of ADZYNMA to treat acute TTP episodes is 40 IU/kg of body weight on Day 1, 20 IU/kg of body weight on Day 2 and 15 IU/kg of body weight starting Day 3 once daily until two days after the acute event is resolved. [1]

✓ 1 In clinical studies, single doses up to 160 IU/kg were used and their safety profile was generally consistent with results from clinical study results in cTTP patients. In case of overdose, based on the pharmacological action of apadamtase alfa, there is the potential for increased risk of bleeding.

Important dosage and administration instructions: [1]

✓ For intravenous use after reconstitution only.
✓ The dose is calculated based on patient body weight.

Home or self-administration under the supervision of a healthcare professional may be considered for patients who are tolerating their injections well^* [1]

In a cross-sectional cohort study with 307 patients (including 25 with cTTP), survey responses indicated that 137 patients (44.6%) would prefer to receive treatment at home; 125 patients (40.7%) would prefer either a doctor or a nurse to administer their treatment at home. [2]

In a cross-sectional cohort study with 307 patients (including 25 with cTTP), survey responses indicated that 137 patients (44.6%) would prefer to receive treatment at home; 125 patients (40.7%) would prefer either a doctor or a nurse to administer their treatment at home. [2]

For further information about ADZYNMA home or self-administration, please refer to the Summary of Product Characteristics.

ADZYNMA Reconstitution and Administration video

See exactly how ADZYNMA is reconstituted and administered by watching this short video.
 

ADZYNMA Storage [1]

Special precautions for storage [1]

✓ Store in a refrigerator (2–8 °C).
✓ Do not freeze.
✓ Store in the original package in order to protect from light.
✓ ADZYNMA may be stored at room temperature up to 30 °C for a period of up to 6 months in lyophilized form, but not exceeding the expiry date.
✓ Do not return ADZYNMA to refrigerated storage after storage at room temperature.
✓ Record on the carton the date ADZYNMA is removed from refrigeration.
✓ Shelf life: 3 years.

Storage conditions after reconstitution of the medicinal product [1]

✓ Chemical and physical in-use stability has been demonstrated for 6 hours at 25 °C.
✓ From a microbiological point of view, unless the method of opening/reconstituting/dilution precludes the risks of microbial contamination, the product should be used immediately. If not used immediately, in-use storage times and conditions are the responsibility of the user.