DESCRIPTION
Imiglucerase is a recombinant analog of the human enzyme β-glucocerebrosidase, a lysosomal glycoprotein enzyme, which normally catalyzes the hydrolysis of glucocerebroside to glucose and ceramide. Mutations of the gene encoding β-glucocerebrosidase prevent this breakdown and lead to accumulation of massive amounts of glucocerebrosides in cells of the macrophage-monocyte system. This condition is known as Gaucher disease, the most common lysosomal storage disease.
Three types of Gaucher disease are delineated by absence or presence and progression of neurologic involvement. Type I disease accounts for 99% of cases and is known as the chronic non-neuronopathic form. Its glucocerebrosidase storage is limited to phagocytes throughout the body although are seen primarily in the spleen and bone marrow. Type II or acute neuronopathic Gaucher disease, is the infantile acute cerebral pattern of the disease dominated by progressive central nervous system disease. Type III disease combines types I and II, with systemic glucocerebrosidase storage throughout the body as well as progressive central nervous system disease. Of the three types, only type I disease is treatable through recombinant enzyme replacement therapy
An example of a preparation of imiglucerase is CerezymeŽ.
REFER TO DECISION SUPPORT TREE
POLICY
Imiglucerase for the treatment of Gaucher disease is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Imiglucerase for the treatment of other conditions/diseases, including, but not limited to, the following is considered investigational:
Type II Gaucher disease
Type III Gaucher disease
MEDICAL APPROPRIATENESS
Imiglucerase for the treatment of Gaucher disease is considered medically appropriate if ALL the following criteria are met:
Disease is classified as Type I
Disease is symptomatic with ANY ONE of the following:
Moderate-to-severe anemia
Thrombocytopenia with bleeding tendency
Bone disease (e.g., chronic bone pain, osteonecrosis, osteopenia, secondary pathologic fractures)
Significant hepatomegaly
Significant splenomegaly
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
Tennessee State law requires coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is relative to life-threatening illnesses, such as cancer, AIDS, and coronary heart disease and recognized in one of the standard reference compendia (As defined in the statute: The United States Pharmacopoeia Drug Information, The American Medical Association Drug Evaluations, & The American Hospital Formulary Service Drug Information) or in the medical literature. This law is applicable to all fully insured members. The law is not applicable to self-funded accounts, but coverage for off-label uses may be provided based on the contractual agreement.
IMPORTANT REMINDER
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
ADDITIONAL INFORMATION
For appropriate dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., The American Hospital Formulary Service Drug Information).
The published literature contained no controlled studies to validate the use of imiglucerase in the treatment/prevention of any other conditions/diseases.
SOURCES
Lexi-Comp, Inc. (2010). AHFS DI. Imiglucerase. Retrieved September 28, 2010 from Lexi-Comp Online with AHFS.
MICROMEDEX Healthcare Series. Drugdex Drug Evaluations. (2010). Imiglucerase. Retrieved September 28, 2010 from MICROMEDEX Healthcare Series.
U. S. Food and Drug Administration. (2005, March). Center for Drug Evaluation and Research. Label and Approval History. CerezymeŽ (imiglucerase for injection). Retrieved September 28, 2010 from http://www.fda.gov/cder/foi/label/2005/20367s066lbl.pdf.
ORIGINAL EFFECTIVE DATE: 8/1/2004
MOST RECENT REVIEW DATE: 11/30/2010
ID_BT
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
Pharmaceutical Decision Support Tree
Imiglucerase (CerezymeŽ)
Is the requested medication being used to treat Type 2 or Type 3 Gaucher's disease?
If yes, this does not meet medical necessity and/or medical appropriateness criteria
If no, go to question #2
Does the individual have a diagnosis of Type I Gaucher's disease?
If yes, go to question #3
If no, this does not meet medical necessity and/or medical appropriateness criteria
Does the individual have symptoms of ANY ONE of the following?
Moderate-to-severe anemia
Thrombocytopenia with bleeding tendency
Bone disease (e.g., chronic bone pain, osteonecrosis, osteopenia, secondary pathologic fractures)
Significant hepatomegaly
Significant splenomegaly
If yes, this satisfies medical necessity and medical appropriateness criteria
If no, this does not meet medical necessity and/or medical appropriateness criteria
This document has been classified as public information.