Criteria
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the
www.fepblue.org
website.
ADAMTS13, recombinant-krhn (Adzynma) may be considered medically necessary when ALL of the following are met:
-
The individual is at least two (2) years of age;
and
-
The individual has not been diagnosed with other Congenital Thrombotic Thrombocytopenic Purpura (cTTP) like disorders (e.g., acquired TTP, immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia-ITP, Evans Syndrome, etc.);
and
-
The individual does not have a medical history or the presence of functional ADAMTS13 inhibitors prior to the start of therapy;
and
-
The individual does not have a known sensitivity to hamster protein;
and
-
The individual has a documented diagnosis of severe hereditary ADAMTS13 deficiency, defined as confirmed by molecular genetic testing, documenting biallelic pathogenic variants in
ADAMTS13
;
and
-
The individual has an ADAMTS13 activity of less than 10 % as measured by the fluorescent resonance energy transfer- von Willebrand factor73 (FRETS-VWF73) assay
(Note: Individuals currently receiving prophylactic plasma infusion therapy may exceed 10% ADAMTS13 activity at start of therapy)
;
and
-
ONE of the following:
-
BOTH of the following:
- Treatment will be used as prophylactic therapy; and
-
The individual has a history of at least one TTP event or is currently receiving prophylactic plasma infusion therapy
(Note: Individuals who have been receiving prophylactic plasma-based therapies should discontinue routine use of those therapies after achieving a therapeutic response)
;
or
- Treatment will be used as on-demand therapy and the individual is at risk of a disease exacerbation.
Reauthorization Criteria
Continuation of therapy with ADAMTS13, recombinant-krhn (Adzynma) may be considered medically necessary when the following criteria are met:
-
The individual continues to meet the universal and other indication-specific relevant criteria identified in section III;
and
-
There is an absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: severe hypersensitivity or anaphylactic reactions, etc.;
and
-
The individual has responded to therapy compared to pre-treatment baseline with the following:
-
If symptomatic, improvement in the signs and symptoms of disease (e.g., neurological symptoms including confusion, dysphonia, dysarthria, focal or general motor symptoms, seizures; renal dysfunction, TTP-related pain, etc.);
and
-
If used for prophylaxis, then one of the following:
-
The individual
has a reduction in or an absence of an acute TTP event, defined by a drop in platelet count (greater than or equal to 50% of baseline or a platelet count less than 100,000/μL) and an elevation of lactate dehydrogenase (LDH) (greater than 2× baseline or greater than 2× upper limit normal (ULN));or
-
The individual has a reduction in or an absence of a sub-acute TTP event, defined by a thrombocytopenia event or a microangiopathic hemolytic anemia event; and organ-specific signs and symptoms including but not limited to renal dysfunction events, neurological symptoms events, fever, fatigue/lethargy, and/or abdominal pain;
or
-
If used for on-demand, the individual has responded to an acute TTP event with therapy as evidenced by improvement in thrombocytopenia
(defined as a drop in platelets greater than or equal to 25% of baseline or a platelet count less than 150,000/mcg/L)
or in microangiopathic hemolytic anemia
(defined as an elevation of LDH greater than 1.5 times of baseline or greater than 1.5 times ULN)
*
Note
: The cumulative amount of medication the individual has on-hand, indicated for the acute treatment of TTP events, will be considered for authorizations.
The authorization will provide a sufficient quantity in order for the individual to have a cumulative amount of medication on-hand in order to treat up to four (4) acute attacks per four (4) weeks for the duration of the authorization (unless otherwise specified).
Procedure Codes
