Criteria
Coverage is subject to the specific terms of the member's benefit plan.
ADAMTS13, recombinant-krhn (Adzynma) may be considered medically necessary for adult and pediatric individuals when the following criteria are met:
- Confirmed diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP) suggested by the following
-
Deficiency in ADAMTS13 enzyme determined by genetic testing;
and
-
Measured ADAMTS13 activity less than 10% (may exceed 10% if individual is receiving prophylactic plasma infusion therapy);
and
-
Treatment is utilized for
ONE
of the following:
- Prophylactic enzyme replacement therapy:
-
Individual does not display signs of severe TTP defined by
BOTH
of the following:
-
Platelet count less than 100,000/mcL;
and
- Elevation of lactate dehydrogenase (LDH) greater than two (2) times the upper limit of normal (ULN);or
- On- demand enzyme replacement therapy:
-
Individual displays signs of severe TTP defined by
BOTH
of the following:
-
Platelet count less than 100,000/mcL or greater than or equal to 50% drop in platelet count;
and
-
Elevation of lactate dehydrogenase (LDH) greater than two (2) times the upper limit of normal (ULN) or greater than two (2) times baseline;
and
- Prescribed or in consultation with a hematologist, oncologist, intensive care specialist, or specialist in rare genetic hematologic diseases; and
- Individual has not been diagnosed with any other TTP-like disorder (microangiopathic hemolytic anemia), including acquired TTP.
Reauthorization Criteria
Continuation of therapy with ADAMTS13, recombinant-krhn (Adzynma) may be considered medically necessary when the following criteria are met:
-
Prescribed or in consultation with a hematologist, oncologist, intensive care specialist, or specialist in rare genetic hematologic diseases;
and
- Individual has demonstrated a positive clinical response documented by reduction in acute or subacute TTP events, improvement in TTP manifestations, platelet counts, microangiopathic hemolytic anemia episodes, or clinical symptoms (i.e. confusion, seizures, renal dysfunction, etc.).
The use of ADAMTS13, recombinant-krhn (Adzynma)
for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
Procedure Code
