Adzynma (ADAMTS13, recombinant-krhn)
Agents to treat Thrombotic Thrombocytopenic Purpura (TTP) Intravenous Kit
Last reviewed · Source: First Databank (FDB) drug data
Overview
ADAMTS13, recombinant-krhn is a human recombinant form of the endogenous ''A disintegrin and metalloproteinase with thrombospondin motifs 13'' (rADAMTS13) enzyme.
ADAMTS13, recombinant-krhn is a recombinant form of the endogenous ADAMTS13 enzyme that is deficient in patients with congenital thrombotic thrombocytopenic purpura. ADAMTS13 is a plasma zinc metalloprotease that regulates the activity of von Willebrand factor (vWF) by cleaving large and ultra-large vWF multimers to smaller units and thereby reducing the platelet binding proteins of vWF and their propensity to form microthrombi. VWF antigen (VWF:AG) and VWF:ristocetin cofactor activity (VWF:RCo) were used to assess the vWF platelet binding activity of ADAMTS13, recombinant-krhn.
Following IV doses of ADAMTS13, recombinant-krhn at the recommended dose, both VWF:AG and VWF:RCo transiently decreased for 1-2 days with a 15-25% change from baseline.
ADAMTS13, recombinant-krhn exhibits dose-proportional pharmacokinetics between the doses of 5 and 40 IU/kg; ADAMTS13 activity appears to reach a maximum in 1 hour post-infusion or earlier. At a dose of 40 IU/kg, the mean half-life in adults was 47.8 hours.
USES: This medication is used to treat a certain blood disorder (congenital thrombotic thrombocytopenic purpura-cTTP). People with cTTP do not have enough of a certain enzyme (ADAMTS13) in the blood, so blood clots form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and limit or stop blood flow to organs such as the brain, kidneys, and heart.
ADAMTS13 works by causing your body to make fewer/smaller blood clots.
Generic name: ADAMTS13, recombinant-krhn
Drug class: Agents to treat Thrombotic Thrombocytopenic Purpura (TTP)
Therapeutic class: Hematological Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Adzynma
Indications
The following indications are listed for Adzynma:
- Congenital Thrombotic Thrombocytopenic Purpura
Also known as: Congenital Adamts-13 Deficiency, Upshaw-Schulman Syndrome.
Dosing
Dosage of ADAMTS13, recombinant-krhn is expressed in IU. The actual ADAMTS13, recombinant-krhn potency in IU is printed on the label of each ADAMTS13, recombinant-krhn vial and carton.
Dosing of ADAMTS13, recombinant-krhn is based on body weight.
No dosing information is available for this tab.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Adzynma 500 Unit Vial | Maintenance | Infuse 40 unit/kg by intravenous route every 2 weeks. |
| Adzynma 1,500 Unit Vial | Maintenance | Infuse 40 unit/kg by intravenous route every 2 weeks. |
| Adzynma 500 Unit Kit | Maintenance | Infuse 40 unit/kg by intravenous route every 2 weeks. |
| Adzynma 1,500 Unit Kit | Maintenance | Infuse 40 unit/kg by intravenous route every 2 weeks. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been conducted with ADAMTS13, recombinant-krhn.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*History of life threatening hypersensitivity reactions to ADAMTS13, recombinant-krhn or its components.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- No disease contraindications
Adverse reactions
The most common adverse reactions (>5%) reported with ADAMTS13, recombinant-krhn in clinical studies were headache, diarrhea, migraine, abdominal pain, nausea, upper respiratory tract infection, dizziness, and vomiting.
Less Frequent
- Anaphylaxis
More Frequent
- Acute abdominal pain
- Diarrhea
- Dizziness
- Headache disorder
- Migraine
- Nausea
- Upper respiratory infection
- Vomiting
Precautions
No enhanced messaging available.
The safety and effectiveness of ADAMTS13, recombinant-krhn have been established in pediatric patients. Clinical trials included patients 2 years of age and older. Based on results from a population pharmacokinetics analysis, no dosage adjustment is required for pediatric patients. The recommended body weight-based dosing regimen in pediatric patients is the same as that in adults.
There are no adequate data regarding the use of ADAMTS13, recombinant-krhn in pregnant women. In clinical studies, 4 patients were exposed to ADAMTS13, recombinant-krhn during pregnancy. One patient experienced a miscarriage that was found to be unrelated to ADAMTS13, recombinant-krhn, 2 patients delivered healthy babies with no safety concerns, and one patient delivered at week 29 with no adverse events believed to be due to ADAMTS13, recombinant-krhn.
ADAMTS13, recombinant-krhn administered in female rats at 80, 200, or 400 international units (IU)/kg every third day from 2 weeks before mating through day 16 of gestation was not associated with any adverse maternal findings or treatment-related effects on embryo-fetal development.
The decision whether ADAMTS13, recombinant-krhn should be used in pregnancy should balance the potential benefits with risks.
It is not known whether ADAMTS13, recombinant-krhn is distributed into human milk, or if the drug has any effects on the breastfed infant or on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for ADAMTS13, recombinant-krhn and any potential adverse effects to the breastfed child.
No dosage adjustment is required in geriatric patients.
Clinical studies of ADAMTS13, recombinant-krhn did not include patients 65 years of age and older to determine whether they respond differently from younger patients. No dosage adjustment is required for elderly patients based on results from a population pharmacokinetic analysis.
ICD codes
| Code | Type | Description |
|---|---|---|
| Congenital thrombotic thrombocytopenic purpura | ||
| D69.42 | ICD10CM | Congenital and hereditary thrombocytopenia purpura |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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