Please wait while the formulary information is being retrieved.
Drug overview for ADZYNMA (adamts13, recombinant-krhn):
Generic name: ADAMTS13, recombinant-krhn
Drug class: Agents to treat Thrombotic Thrombocytopenic Purpura (TTP)
Therapeutic class: Hematological Agents
ADAMTS13, recombinant-krhn is a human recombinant form of the endogenous ''A disintegrin and metalloproteinase with thrombospondin motifs 13'' (rADAMTS13) enzyme.
No enhanced Uses information available for this drug.
Generic name: ADAMTS13, recombinant-krhn
Drug class: Agents to treat Thrombotic Thrombocytopenic Purpura (TTP)
Therapeutic class: Hematological Agents
ADAMTS13, recombinant-krhn is a human recombinant form of the endogenous ''A disintegrin and metalloproteinase with thrombospondin motifs 13'' (rADAMTS13) enzyme.
No enhanced Uses information available for this drug.
DRUG IMAGES
No Image Available
The following indications for ADZYNMA (adamts13, recombinant-krhn) have been approved by the FDA:
Indications:
Congenital thrombotic thrombocytopenic purpura
Professional Synonyms:
Congenital ADAMTS-13 deficiency
Upshaw-Schulman syndrome
Indications:
Congenital thrombotic thrombocytopenic purpura
Professional Synonyms:
Congenital ADAMTS-13 deficiency
Upshaw-Schulman syndrome
The following dosing information is available for ADZYNMA (adamts13, recombinant-krhn):
It isessential that the manufacturer's labeling be consulted for more detailed information on dosage and administration of this drug. Dosage summary:
For IV use after reconstitution only.
Each vial is labeled with the actual rADAMTS13 activity, measured in terms of its potency in International Units (IU). Calculate administration dose and volume based on the patient's body weight using the actual potency (and not the nominal potency) as printed on vial.
The recommended body-weight based dosing regimen in pediatric patients is the same as that in adults.
See Full Prescribing Information for specific instructions on preparation and administration.
For IV use after reconstitution only.
Each vial is labeled with the actual rADAMTS13 activity, measured in terms of its potency in International Units (IU). Calculate administration dose and volume based on the patient's body weight using the actual potency (and not the nominal potency) as printed on vial.
The recommended body-weight based dosing regimen in pediatric patients is the same as that in adults.
See Full Prescribing Information for specific instructions on preparation and administration.
No enhanced Administration information available for this drug.
| DRUG LABEL | DOSING TYPE | DOSING INSTRUCTIONS |
|---|---|---|
| ADZYNMA 500 UNIT VIAL | Maintenance | Adults infuse 40 unit/kg by intravenous route every 2 weeks |
| ADZYNMA 1,500 UNIT VIAL | Maintenance | Adults infuse 40 unit/kg by intravenous route every 2 weeks |
| ADZYNMA 500 UNIT KIT | Maintenance | Adults infuse 40 unit/kg by intravenous route every 2 weeks |
| ADZYNMA 1,500 UNIT KIT | Maintenance | Adults infuse 40 unit/kg by intravenous route every 2 weeks |
No generic dosing information available.
The following drug interaction information is available for ADZYNMA (adamts13, recombinant-krhn):
There are 0 contraindications.
There are 0 severe interactions.
There are 0 moderate interactions.
The following contraindication information is available for ADZYNMA (adamts13, recombinant-krhn):
Drug contraindication overview.
Do not use in patients who have manifested life threatening hypersensitivity reactions to ADAMTS13, recombinant-krhn or its components.
Do not use in patients who have manifested life threatening hypersensitivity reactions to ADAMTS13, recombinant-krhn or its components.
There are 0 contraindications.
There are 0 severe contraindications.
There are 1 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| No disease contraindications |
The following adverse reaction information is available for ADZYNMA (adamts13, recombinant-krhn):
Adverse reaction overview.
Most common adverse reactions (incidence >5%) are headache, diarrhea, migraine, abdominal pain, nausea, upper respiratory tract infection, dizziness, and vomiting.
Most common adverse reactions (incidence >5%) are headache, diarrhea, migraine, abdominal pain, nausea, upper respiratory tract infection, dizziness, and vomiting.
There are 1 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
| None. |
Anaphylaxis |
| Rare/Very Rare |
|---|
| None. |
There are 8 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Acute abdominal pain Diarrhea Dizziness Headache disorder Migraine Nausea Upper respiratory infection Vomiting |
None. |
| Rare/Very Rare |
|---|
| None. |
The following precautions are available for ADZYNMA (adamts13, recombinant-krhn):
The safety and effectiveness of ADAMTS13, recombinant-krhn has been established in pediatric patients. Clinical trials included patients 2 years of age and older. Based on data from population pharmacokinetic (PK) analysis, no additional dose adjustments beyond body weight are required for this age group. The recommended body-weight based dosing regimen in pediatric patients is the same as that in adults.
Contraindicated
Severe Precaution
Management or Monitoring Precaution
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
The safety of ADAMTS13, recombinant-krhn for use during pregnancy has not been established in controlled clinical trials. Limited data with use during pregnancy are insufficient to inform a drug-associated risk of adverse developmental outcomes. In determining whether ADAMTS13, recombinant-krhn should be used in pregnancy, healthcare providers should balance the potential benefits with the potential risks.
The background rate of major birth defects and miscarriage in the indicated population is unknown. In the U.S.
general population, the estimated background rate of major birth defects and miscarriage in clinically recognized pregnancies is 2% to 4% and 15% to 20%, respectively. There have been four cTTP patients exposed to ADAMTS13, recombinant-krhn during pregnancy. Two patients in a long-term extension study were found to be pregnant early in the first trimester while receiving prophylaxis with ADAMTS13, recombinant-krhn.
Both patients were discontinued from the study to comply with the protocol requirements. The first patient had no further exposure to ADAMTS13, recombinant-krhn and had a first trimester miscarriage approximately two months after study discontinuation. The investigator assessed the event was unrelated to ADAMTS13, recombinant-krhn.
The second patient resumed treatment with ADAMTS13, recombinant-krhn under a compassionate use program and delivered a healthy full-term baby with no safety concerns reported by the investigator. Two additional cTTP patients were treated with ADAMTS13, recombinant-krhn in a compassionate use program during pregnancy. The first patient, in the third trimester of her second pregnancy, experienced a stroke and thrombocytopenia that was refractory to daily plasmapheresis.
At 33 weeks of gestation, ADAMTS13, recombinant-krhn treatment was started once weekly. ADAMTS13 activity levels normalized, thrombocytopenia resolved, and a healthy baby was delivered at 37 weeks with no safety concerns reported by the treating physician due to ADAMTS13, recombinant-krhn. The second patient had an exacerbation of her cTTP during her second trimester of pregnancy despite prior daily plasma exchange.
Her pregnancy was considered to be at risk, with inadequate response to plasma-based therapies. ADAMTS13, recombinant-krhn was started once weekly and induced clinical remission. The baby was delivered by a cesarean section at week 29 and the treating physician reported no adverse events due to ADAMTS13, recombinant-krhn. Whether ADAMTS13, recombinant-krhn should be used in pregnancy is solely up to the medical judgment of the healthcare provider.
The background rate of major birth defects and miscarriage in the indicated population is unknown. In the U.S.
general population, the estimated background rate of major birth defects and miscarriage in clinically recognized pregnancies is 2% to 4% and 15% to 20%, respectively. There have been four cTTP patients exposed to ADAMTS13, recombinant-krhn during pregnancy. Two patients in a long-term extension study were found to be pregnant early in the first trimester while receiving prophylaxis with ADAMTS13, recombinant-krhn.
Both patients were discontinued from the study to comply with the protocol requirements. The first patient had no further exposure to ADAMTS13, recombinant-krhn and had a first trimester miscarriage approximately two months after study discontinuation. The investigator assessed the event was unrelated to ADAMTS13, recombinant-krhn.
The second patient resumed treatment with ADAMTS13, recombinant-krhn under a compassionate use program and delivered a healthy full-term baby with no safety concerns reported by the investigator. Two additional cTTP patients were treated with ADAMTS13, recombinant-krhn in a compassionate use program during pregnancy. The first patient, in the third trimester of her second pregnancy, experienced a stroke and thrombocytopenia that was refractory to daily plasmapheresis.
At 33 weeks of gestation, ADAMTS13, recombinant-krhn treatment was started once weekly. ADAMTS13 activity levels normalized, thrombocytopenia resolved, and a healthy baby was delivered at 37 weeks with no safety concerns reported by the treating physician due to ADAMTS13, recombinant-krhn. The second patient had an exacerbation of her cTTP during her second trimester of pregnancy despite prior daily plasma exchange.
Her pregnancy was considered to be at risk, with inadequate response to plasma-based therapies. ADAMTS13, recombinant-krhn was started once weekly and induced clinical remission. The baby was delivered by a cesarean section at week 29 and the treating physician reported no adverse events due to ADAMTS13, recombinant-krhn. Whether ADAMTS13, recombinant-krhn should be used in pregnancy is solely up to the medical judgment of the healthcare provider.
There is no information regarding the presence of ADAMTS13, recombinant-krhn in human milk, its effects on milk production or the breastfed infant. 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.
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. Based on the results from population pharmacokinetics analysis, no dose adjustment is required for elderly patients.
The following prioritized warning is available for ADZYNMA (adamts13, recombinant-krhn):
No warning message for this drug.
No warning message for this drug.
The following icd codes are available for ADZYNMA (adamts13, recombinant-krhn)'s list of indications:
| Congenital thrombotic thrombocytopenic purpura | |
| D69.42 | Congenital and hereditary thrombocytopenia purpura |
Formulary Reference Tool