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Date Printed: June 26, 2017: 01:14 AM

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09-J0000-06

Original Effective Date: 12/15/99

Reviewed: 07/13/16

Revised: 11/15/16

Subject: Immune Globulin Therapy

THIS MEDICAL COVERAGE GUIDELINE IS NOT AN AUTHORIZATION, CERTIFICATION, EXPLANATION OF BENEFITS, OR A GUARANTEE OF PAYMENT, NOR DOES IT SUBSTITUTE FOR OR CONSTITUTE MEDICAL ADVICE. ALL MEDICAL DECISIONS ARE SOLELY THE RESPONSIBILITY OF THE PATIENT AND PHYSICIAN. BENEFITS ARE DETERMINED BY THE GROUP CONTRACT, MEMBER BENEFIT BOOKLET, AND/OR INDIVIDUAL SUBSCRIBER CERTIFICATE IN EFFECT AT THE TIME SERVICES WERE RENDERED. THIS MEDICAL COVERAGE GUIDELINE APPLIES TO ALL LINES OF BUSINESS UNLESS OTHERWISE NOTED IN THE PROGRAM EXCEPTIONS SECTION.

           
Position Statement Dosage/ Administration Billing/Coding Reimbursement Program Exceptions Definitions
           
Related Guidelines Other References Updates  
           

DESCRIPTION:

Intravenous immune globulin (IVIG) (Carimune NF®, Flebogamma®DIF, Gammagard® Liquid, Gammagard® S/D, Gammagard® S/D Less IgA, Gammaplex®, Gammaked™, Gamunex®-C, Octagam®, Privigen®, and Bivigam®) is an antibody-containing solution obtained from the pooled plasma of healthy blood donors that contains antibodies to greater than 10 million antigens. IVIG has been used to correct immune deficiencies in patients with either inherited or acquired immunodeficiencies and has also been investigated as an immunomodulator in diseases thought to have an autoimmune component. The U.S. Food and Drug Administration (FDA) approved and off-label indications are listed below.

Subcutaneous immune globulin (SCIG) (Cuvitru™, Gammagard® Liquid, Gammaked™, Gamunex®-C, Hizentra®,HyQvia®) are covered under the same criteria as IVIG products.

POSITION STATEMENT:

Comparative Effectiveness

The Food and Drug Administration has deemed the drug(s) or biological product(s) in this coverage policy to be appropriate for self-administration or administration by a caregiver (i.e., not a healthcare professional). Therefore, coverage (i.e., administration) in a provider-administered setting such as an outpatient hospital, ambulatory surgical suite, or emergency facility is not considered medically necessary. This statement applies to Cuvitru™, Hizentra®, and HyQvia®, and the following immune globulin products only when administered subcutaneously: Gammagard® Liquid, Gammaked™, Gamunex®-C. 

I. Initiation of intravenous immune globulin (IVIG) and subcutaneous immune globulin (SCIG) meets the definition of medical necessity when administered for treatment of the indications listed in Table 1 and ALL of the indication-specific criteria are met:

Table 1

Indications and Criteria

Primary Immunodeficiency

Agammaglobulinemia

When ONE of the following criteria is met:

1. Serum IgG level <200 mg/dL

2. Extremely low (<2%) or absent B cell count (CD19+)

Approval duration: 6 months

Ataxia telangiectasia

When BOTH of the following are met:

1. Lack of protective antibody titers*

2. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Common Variable Immune Deficiency (CVID)

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Lack of protective antibody titers*

3. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Functional Immunodeficiency

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Lack of protective antibody titers*

3. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Hyper-IgE syndrome

When BOTH of the following are met:

1. Lack of protective antibody titers*

2. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Hyper-IgM syndrome or CD40 ligand (CD40L) deficiency

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Lack of protective antibody titers*

3. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Hypogammaglobulinemia

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Lack of protective antibody titers*

3. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

IgG subclass deficiency

When ALL of the following are met:

1. Documented deficiency of one or more IgG subclasses§ greater than 2 standard deviations below the age-specific mean (confirmed by 2 measurements at least 1 month apart)

2. Lack of protective antibody titers*

3. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Nuclear factor kappa-B essential modulator (NEMO) syndrome

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Lack of protective antibody titers*

3. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Severe Combined Immunodeficiency Syndrome (SCID)

When ALL of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL OR documented T cells (CD3) are severely low or absent (<300/microL)

2. Lack of protective antibody titers*

3. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Specific antibody deficiency (SAD)

When BOTH of the following are met:

1. Lack of protective antibody titers*

2. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Warts, hypogammaglobulinemia, immunodeficiency, and myelokathexis (WHIM) syndrome

When BOTH of the following criteria are met:

1. Documented serum IgG less than 600 mg/dL

2. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Wiskott-Aldrich Syndrome

Documented serum IgM level two standard deviations (2SD) below the mean level based on age and gender and when ONE of the following is met:

1. Lack of protective antibody titers*

2. Recurrent, difficult to treat bacterial infections

Approval duration: 6 months

Secondary Immunodeficiency

High-risk, preterm, low-birth-weight neonates

Prevention or adjunct treatment for infection

Approval duration: 3 months

HIV-infected children

When used for prevention of bacterial infection and ALL of the following are met:

1. Member is 13 years of age or less

2. CD4+ count is greater than 200/µL

3. IVIG will be used in conjunction with antiretroviral treatment

4. Member’s IgG level is less than 400 mg/dL

Approval duration: 6 months

Acquired hypogammaglobulinemia conditions including:

• Chronic Lymphocytic Leukemia (CLL)

• Acute Lymphocytic (lymphoblastic) Leukemia (ALL)

• Acute Myelogenous Leukemia (AML)

• Chronic Myelogenous Leukemia (CML)

• Multiple Myeloma (MM)

• Non-Hodgkin’s Lymphoma

• Extensive burns

• Collagen-vascular disease

Documented serum IgG level less than 500 mg/dL on 2 or more occasions and when one of the following is met:

1. Lack of protective antibody titers*

2. Recurrent difficult to treat bacterial infections

Approval duration: 6 months

Allogeneic hematopoietic stem cell transplant (HSCT) or bone marrow transplantation (BMT)

HSCT or BMT when used for prevention of infection and either of the following criteria are met:

1. First 100 days post-transplant,

2. More than 100 days post-transplant and one of the following is met:

a. Viral infection (e.g., CMV, EBV, RSV)

b. Serum IgG level less than 400 mg/dL

BMT when used for graft-versus-host disease (GVHD)and ALL of the following criteria are met:

1. Corticosteroid-resistant GVHD

2. First 100 days post-transplant

3. Serum IgG level less than 400 mg/dL

Approval duration: 6 months

Solid organ transplants

Allosensitized† members awaiting solid organ transplant

Approval duration: 3 months

Hematology

Acute idiopathic thrombocytopenic purpura (ITP)

Treatment of children (i.e.,18 years of age or less) when ANY of the following criteria are met:

1. Member’s platelet (PLT) count is less than 20,000

2. Member’s PLT count is less than 30,000 and the member has an active bleed

3. Member’s PLT count is less than 100,000 and the member is scheduled to undergo a major surgical procedure (e.g., splenectomy)

Treatment of adults (i.e., greater than 18 years of age) when EITHER of the following criteria are met:

1. Member’s PLT count is less than 30,000

2. Member’s PLT count is less than 100,000 and the member is scheduled to undergo a major surgical procedure (e.g., splenectomy)

Approval duration: 6 months

Chronic ITP

Treatment when ALL of the following criteria are met:

1. Duration greater than 6 months

2. Member has tried/failed or has a contraindication to corticosteroid treatment

3. Member’s platelet count is less than 30,000

4. Other causes of thrombocytopenia (e.g., concurrent illness/disease) have been ruled out

Approval duration: 1 year

HCV-associated thrombocytopenia

Treatment when ALL of the following criteria met:

1. Member’s platelet count is less than 30,000

2. ITP is refractory to antiviral therapy or member has contraindication to antivirals

Approval duration: 6 months

HIV-associated thrombocytopenia

Treatment when ALL of the following criteria are met:

1.Member’s platelet count is less than 30,000

2.ITP is refractory to antiretroviral therapy or member has contraindication to antiretrovirals (e.g., high dose zidovudine monotherapy or highly active antiretroviral therapy [HAART])

3. Member has tried/failed or has a contraindication to corticosteroid treatment

Approval duration: 6 months

Fetal or neonatal Alloimmune Thrombocytopenia (FAIT, NAIT)

Treatment of ante-natal FAIT/NAIT when both of the following criteria are met:

1. Prior FAIT birth

2. Detectable maternal antibodies to paternal platelet antigen are present

Approval duration 1 year

Treatment of post-natal FAIT/NAIT when ALL of the following criteria are met:

1. Other causes of thrombocytopenia have been ruled out (e.g., infection, disseminated intravascular coagulation)

2. Member’s platelet count is less than 50,000

3. Detectable maternal antibodies to paternal platelet antigen are present

4. Thrombocytopenia persists after transfusion of anti-negative compatible platelets

Approval duration: 6 months

ITP in pregnancy

Treatment when EITHER of the following criteria are met:

1. Member’s PLT count is less than 50,000

2. History of splenectomy

Approval duration: 1 year

Post-transfusion purpura**

Acute treatment only (i.e., IVIG is administered within 2-14 days post-transfusion)

Approval duration: 30 days

Neonatal isoimmune hemolytic disease**

When used for acute treatment in conjunction with phototherapy

Approval duration: 30 days

Warm antibody autoimmune hemolytic anemia (wAIHA)

Treatment when ALL of the following criteria are met:

1. wAIHA is confirmed by a positive direct Coombs test for immunoglobulin G(IgG), complement (C3d), or both

2. Member has contraindication to or tried/failed an adequate trial of corticosteroids (e.g., prednisone 1 mg/kg for 3 weeks) and immunosuppressants (e.g. azathioprine, cyclosporine, cyclophosphamide)

3. The member has relapsed following splenectomy or is not a candidate for splenectomy

Approval duration: 1 year

Evan’s Syndrome

Documented treatment failure, contraindication, intolerance to conventional therapy (e.g., azathioprine, cyclophosphamide, cyclosporine, prednisone)

Approval duration: 1 year

Neurology

Acute treatment of Myasthenia gravis**

Treatment when ANY of the following criteria are met:

1. Acute crisis (<5 days treatment) with decompensation (e.g., respiratory failure, inability to perform physical activity)

2. During or prior to initiation of immunosuppressive therapy to prevent disease exacerbation

3. Prior to thymectomy for a member with significant bulbar dysfunction

Approval duration: 5 days

Refractory Myasthenia gravis

When the member has progressive disease with documented failure, contraindication, or intolerance to ALL of the following:

1. pyridostigmine

2. corticosteroids

3. azathioprine

4. cyclosporine

Approval duration: 6 months

Chronic inflammatory demyelinating polyneuropathy (CIDP)

Treatment when ALL of the following criteria are met:

1. Member’s clinical course is relapsing and remitting or progressive for more than 2 months

2. Member’s disease has been confirmed by electrophysiologic findings that demonstrate any 3 of the following

a. Partial conduction block of 1 or more motor nerves

b. Reduced conduction velocity of 2 or more motor nerves

c. Prolonged distal latency of 2 or more motor nerves

d. Prolonged F-wave latencies of 2 or more nerves or the absence of F-waves

3. Member’s disease has been confirmed by BOTH of the following physiologic findings

a. Hypo- or areflexia

b. Motor or sensory impairment of more than one limb

Approval Duration: 1 year

Multifocal Motor Neuropathy (MMN)

Treatment when the following criteria are met:

1. Member’s disease has been confirmed by electrophysiologic findings including BOTH of the following

a. Presence of either

­ Probable conduction block in at least two motor nerve segments

­ Definite conduction block in at least one motor nerve segment and probable conduction block in a different motor nerve segment

b. Normal results for sensory nerve conduction on all tested nerves

2. Progressive symptoms are present for one or more months

Approval duration: 1 year

Relapsing-Remitting Multiple Sclerosis (RRMS)

Treatment when BOTH of the following are met:

1. Member has tried and failed, or has a contraindication to THREE or more of the following:

a. interferon beta-1a (Avonex or Rebif)

b. interferon beta-1b (Betaseron, Extavia)

c. fingolimod (Gilenya)

d. glatiramer acetate (Copaxone, Glatopa)

e. dimethyl fumarate (Tecfidera)

f. natalizumab (Tysabri)

g. peg-interferon beta-1a (Plegridy™)

h. teriflunomide (Aubagio)

2. IVIG will be used as monotherapy in the treatment of RRMS

Approval duration: 1 year

Guillian-Barré Syndrome (GBS)- Acute inflammatory demyelinating neuropathy (AIDP)

Acute treatment when ALL of the following criteria are met:

1. Member has severe disease (e.g., is unable to walk)

2. Onset of symptoms occurred within the last 4 weeks

3. No concomitant plasma exchange therapy

Approval duration: 1 year

Lambert-Eaton Myasthenic Syndrome (LEMS)

Documented treatment failure, contraindication, or intolerance to available standard therapy (e.g., acetyl cholinesterase inhibitors, prednisone, and azathioprine).

Approval duration: 1 year

Stiff Person Syndrome (Moersch-Woltmann Syndrome)

Documented treatment failure, contraindication, or intolerance to available standard medication therapy (e.g., diazepam, baclofen, phenytoin, clonidine, or tizanidine).

Approval duration: 1 year

Rheumatic Disorders

Dermatomyositis or Polymyositis

Treatment when ALL of the following criteria are met:

1. Diagnosis confirmed by muscle biopsy

2. Failure or contraindication to corticosteroids (e.g., prednisone)

3. Failure or contraindication to immunosuppressants (e.g., azathioprine, methotrexate, cyclophosphamide)

Approval duration: 1 year

Kawasaki Disease**

Acute treatment (within 10 days of onset of symptoms) when given in conjunction with aspirin

Approval duration: 3 months

Infectious Disease

Staphylococcal or streptococcal Toxic Shock Syndrome**

Acute treatment when one of the following is met:

1. Infection refractory to aggressive treatment

2. Presence of an undrainable focus

3. Persistent oliguria with pulmonary edema

Approval duration: 30 days

Measles post-exposure prophylaxis**

When one of the following is met:

1.Member is immunocompromised (HIV, transplant, etc).

2.Member is pregnant without evidence of measles immunity

Approval duration: 3 months

Maternal-fetal transmission of HIV in women who are in their third trimester of pregnancy**

When used in conjunction with antiretroviral treatment

Approval duration: 4 months

CMV pneumonia**

When all of the following are met:

1. Member is immunocompromised

2. Infection refractory to standard treatment

3. Therapy is in combination with ganciclovir or foscarnet

Appproval duration: 10 days

RSV**

When all of the following are met:

1. Member is immunocompromised

2. Infection refractory to standard treatment

3. Therapy is in combination with ribavirin

Appproval duration: 10 days

Parvovirus B19**

When ALL of the of following are met:

1. Member is immunocompromised

2. Severe anemia associated with bone marrow suppression

Appproval duration: 5 days

Varicella-zoster post-exposure prophylaxis**

When Varicella-zoster immune globulin is unavailable or contraindicated and ONE of the following is met:

1. Member is immunocompromised

2. Member is pregnant without evidence of varicella immunity

3. Member is a neonate exposed at time of delivery

4. Member was exposed during hospitalization and is born premature (>28 weeks gestation) and mother does not have evidence of immunity

5. Member was exposed during hospitalization and is born premature at a low birth weight (<28 weeks gestation and weighs < 1 kg at birth)

Appproval duration: 1 dose

Dermatology

Autoimmune mucocutaneous blistering diseases such as:

• Pemphigus vulgaris

• Pemphigus folacious

• Bullous pemphigoid

• Mucous membrane pemphigoid

• Epidermolysis Bullosa Acquisita

• Stevens-Johnson syndrome

• Toxic epidermal necrolysis (TEN)

Treatment when EITHER of the following criteria are met:

1. Member has tried/failed or has a contraindication to conventional therapy (corticosteroids, azathioprine, cyclophosphamide, or mycophenolate)

2. Member has rapidly progressive disease in which conventional therapy would not achieve a response quickly enough AND IVIG will be initiated along with concurrent conventional therapy.

Approval duration: 6 consecutive months

* Lack of protective antibody titers requires laboratory confirmation of failure to produce antibodies 3 to 4 weeks following tetanus (<0.1 IU/mL) OR failure to produce antibodies 4 to 8 weeks after administration of pneumococcal polysaccharide or pneumococcal conjugate vaccine based on the following measures:

• Age < 6 years, Concentration greater than 1.3 mcg/mL for <50% of serotypes

• Age > 6 years, Concentration greater than 1.3 mcg/mL for <70% of serotypes

** Diagnosis excluded from continuation criteria (i.e.,initiation criteria must be met)

Quest diagnostics can perform the enzyme immunoassay that detects serum or plasma antibodies directed towards HLA class I antigens and platelet specific antigens (HPA-1 through HPA-8).

Quest diagnostics can perform the Direct Coombs test.

§ IgG4 levels excluded

II. Continuation of intravenous (IV), or subcutaneous (SC)immune globulin (including transitioning between products) meets the definition of medical necessity for the indications in Table 1 (exceptions noted) when ALL of the following criteria are met:

1. The member has been previously approved by Florida Blue or another health plan in the past 2 years, OR the member has previously met all indication-specific criteria

2. The member has a beneficial response to therapy – documentation must be provided

3. In clinically appropriate indications, dose is titrated to the minimum effective dose and frequency to sustain clinical response

Approval duration: 1 year

III. Intravenous immune globulin (IVIG) (J1566, J1567, 90283) is considered experimental or investigational for the following conditions (not all-inclusive) due to the lack of clinical data to support the effects of better health outcomes:

• Aplastic anemia

• Adult AIDS

• Asthma

• Autism

• Chronic fatigue syndrome

• Chronic progressive multiple sclerosis

• Chronic sinusitis

• Cystic fibrosis

• Diabetes mellitus

• Diamond blackfan anemia

• Epilepsy (adult or pediatric)

• Hemolytic uremic syndrome

• Inclusion body myositis

• Nonimmune thrombocytopenia

• Other vasculitides, besides Kawasaki disease

• Paraneoplastic syndrome including but not limited to Eaton Lambert syndrome

• Red cell aplasia

• Refractory rheumatoid arthritis and other connective tissue diseases

• Recurrent spontaneous abortion

• Thrombotic thrombocytopenic purpura

• Upper respiratory infection, recurrent

• Prophylaxis of preterm or low birth weight infants without signs or symptoms of infection.

DOSAGE/ADMINISTRATION:

THIS INFORMATION IS PROVIDED FOR INFORMATIONAL PURPOSES ONLY AND SHOULD NOT BE USED AS A SOURCE FOR MAKING PRESCRIBING OR OTHER MEDICAL DETERMINATIONS. PROVIDERS SHOULD REFER TO THE MANUFACTURER’S FULL PRESCRIBING INFORMATION FOR DOSAGE GUIDELINES AND OTHER INFORMATION RELATED TO THIS MEDICATION BEFORE MAKING ANY CLINICAL DECISIONS REGARDING ITS USAGE.

Dosage is highly variable depending on individual response, indication or product selected. Refer to prescribing literature (e.g., package insert, etc.).

Dosing should be calculated using adjusted body weight if one or more of the following criteria are met:

• Patient’s body mass index (BMI) is 30kg/m2 or more; OR

• Patient’s actual body weight is 20% higher than his or her ideal body weight (IBW)

Use the following dosing formulas to calculate the adjusted body weight (round dose to nearest 5 gram increment in adult patients):

Dosing formulas

BMI = 703 x (weight in pounds/height in inches2)

IBW(kg) for males = 50 + [2.3 x (height in inches – 60)]

IBW(kg) for females = 45.5 + [2.3 x (height in inches – 60)]

Adjusted body weight = IBW + 0.5 (actual body weight – IBW)

This information is not meant to replace clinical decision making when initiating or modifying medication therapy and should only be used as a guide. Patient-specific variables should be taken into account.

CONTRAINDICATIONS/PRECAUTIONS

Immune Globulin (IV, SC)

Contraindications

• Black Box Warning:

o IVIG products have been associated with renal dysfunction, acute renal failure, osmotic nephrosis, and death. Use caution in patients predisposed to acute renal failure (age> 65 yrs, use of nephrotoxic drugs, preexisting renal insufficiency, diabetes mellitus, volume depletion, sepsis, paraproteinemia) and administer at the minimum concentration available and the minimum rate of infusion practicable. Renal effects are more common with high sucrose content and high osmolality. Members should be appropriately hydrated prior to administration.

o Thrombosis may occur regardless of the route of administration and in the absence of known risk factors. Risk is increased with advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, estrogen use, indwelling central vascular catheters, hyperviscosity, and cardiovascular risk factors. Administer in patients at risk of thrombosis at the minimum dose and infusion rate practical and ensure adequate hydration prior to therapy Monitor for signs and symptoms of thrombosis and assess blood viscosity in persons at risk for hyperviscosity.

• Hereditary intolerance to fructose, including infants and neonates in whom tolerance to sucrose or fructose has not been established.

• Hyperprolinemia (Type I or II): L-proline contained in Hizentra and Privigen.

• Hypersensitivity to immune globulin or any component of the formulation (including polysorbate 80). Anaphylaxis, inflammatory reactions, characterized by a rise in temperature, chills, nausea, and vomiting, and hypersensitivity reactions may occur.

• Persons with selective IgA deficiency with antibodies against IgA, and a history of hypersensitivity.

Precautions

• Endocrine: Falsely elevated glucose measurements may occur.

• Hematologic: Hemolysis and delayed hemolytic anemia may occur. Severe hemolysis-related renal dysfunction, renal failure and disseminated intravascular coagulation have been reported.

• Infusion reactions: Severe hypersensitivity reactions have been reported and fever, chills, nausea vomiting may occur. Monitoring is recommended and discontinue for severe reactions.

• Immunologic: IVIG products are of human plasma origin and may contain infectious agents.

• Metabolic: Hyperproteinemia, increased serum viscosity and hyponatremia or hypernatremia may occur.

• Neurologic: Aseptic meningitis syndrome may occur with high doses (≥1 gram/kg or rapid infusion).

• Pregnancy: IVIG is classified as Pregnancy risk category C. No complications to the fetus have been reported, but it has not been well studied in pregnant women.

• Renal: Acute renal dysfunction can rarely occur, usually within seven days of use. Avoid use in members with CrCl < 10 ml/min. Use caution in elderly and those with renal disease, diabetes, sepsis, volume depletion, concomitant nephrotoxic agents, etc., due to the risk of renal dysfunction. Consider infusion at a rate less than maximum. Baseline renal function should be assessed prior to starting IVIG and periodically during administrations. Ensure that members are well-hydrated prior to therapy. If renal function worsens, consider discontinuing therapy or using products that do not contain sucrose (e.g. Gamunex).

• Respiratory: Transfusion-related acute lung injury may occur.

• Subcutaneous administration: Not recommended for ITP due to increased risk of hematoma. Do not inadvertently infuse subcutaneous form due to increased risk of thrombosis.

• Thrombosis: Use caution in members with a history of thrombotic events or cardiovascular disease. There is clinical evidence of a possible association between thrombotic events (i.e., deep vein thrombosis, myocardial infarction, cerebral vascular accident, etc.) and the administration of IVIG.

• Volume: Expanded fluid volume may cause overload with high-dose regimens for chronic ITP.

BILLING/CODING INFORMATION:

Note: This list of codes may not be all-inclusive.

HCPCS Coding:

C9399

Unclassified drugs or biologicals

J1459

Injection, immune globulin (Privigen), intravenous, non-lyophilized (e.g. liquid), 500 mg

J1556

Injection, immune globulin (BIVIGAM), 500 mg

J1557

Injection, immune globulin, (Gammaplex), intravenous, non-lyophilized (e.g. liquid), 500 mg

J1559

Injection, immune globulin (Hizentra), 100 mg

J1561

Injection, immune globulin, (Gamunex-C, Gammaked), intravenous, non-lyophilized (e.g., liquid), 500mg

J1566

Injection, immune globulin, intravenous, lyophilized (e.g. powder) not otherwise specified, 500 mg (use for Carimune NF, Panglobulin NF, and Gammagard S/D)

J1568

Injection, immune globulin, (Octagam), intravenous, non-lyophilized (e.g., liquid), 500mg

J1569

Injection, immune globulin, (Gammagard liquid), intravenous, non-lyophilized (e.g., liquid), 500mg

J1572

Injection, immune globulin, (Flebogamma/Flebogamma DIF), intravenous, non-lyophilized (e.g., liquid), 500mg

J1575

Injection, immune globulin/hyaluronidase, (hyqvia), 100 mg immune globulin

J1599

Injection, immune globulin, intravenous, non-lyophilized (e.g., liquid), not otherwise specified, 500 mg

J3590

Unclassified biologics

CPT Coding:

90283

Immune Globulin (IgIV), human, for intravenous use

90284

Immune Globulin (SCIg), human, for use in subcutaneous infusions

ICD-10 Diagnoses Codes That Support Medical Necessity (IVIG, SCIG – J1459, J1556, J1557, J1559, J1561, J1566, J1568, J1569, J1572, J1599, 90283, 90284): (Effective 10/01/15)

A48.3

Toxic shock syndrome

B01.0 – B01.89

Varicella

B05.0 – B05.89

Measles

B06.0 – B06.89

Rubella

B18.2

Chronic viral hepatitis C

B20

Human immunodeficiency virus [HIV] disease

B25.0 – B25.9

Cytomegalovirus disease

B27.00 – B27.99

Infectious mononeucleosis (Epstein Barr virus)

B34.3

Parvovirus infection

B97.4

Respiratory syncytial virus

C82 – C85.9

Lymphomas (nonhodgkins)

C90.00

Multiple myeloma not having achieved remission

C90.01

Multiple myeloma in remission

C90.02

Multiple myeloma in relapse

C91.0 – C91.02

Acute lymphoblastic leukemia

C91.10

Chronic lymphocytic leukemia of B-cell type not having achieved remission

C91.11

Chronic lymphocytic leukemia of B-cell type in remission

C91.12

Chronic lymphocytic leukemia of B-cell type in relapse

C92.00 – C92.02
C92.40 – C92.42
C92.50 – C92.52
C92.60 – C92.62
C92.A0 – C92.A2

Acute myeloblastic leukemia

C92.1 – C92.12

Chronic myeloblastic leukemia

D59.0

Drug-induced autoimmune hemolytic anemia

D59.1

Other autoimmune hemolytic anemias

D69.3

Immune thrombocytopenic purpura

D69.41

Evans syndrome

D69.42

Congenital and hereditary thrombocytopenia purpura

D69.49

Other primary thrombocytopenia

D69.51

Posttransfusion purpura

D69.59

Other secondary thrombocytopenia

D69.6

Thrombocytopenia, unspecified

D71

Functional disorder of polymorphonuclear neutrophils

D80.0

Hereditary hypogammaglobulinemia

D80.1

Nonfamilial hypogammaglobulinemia

D80.2

Selective deficiency of immunoglobulin A [IgA]

D80.3

Selective deficiency of immunoglobulin G [IgG] subclasses

D80.4

Selective deficiency of immunoglobulin M [IgM]

D80.5

Immunodeficiency with increased immunoglobulin M [IgM]

D80.6

Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia (Specific antibody deficiency)

D80.7

Transient hypogammaglobulinemia of infancy

D80.8

Other immunodeficiencies with predominant antibody defects

D80.9

Immunodeficiency with predominantly antibody defects, unspecified

D81.0

Severe combined immunodeficiency [SCID] with reticular dysgenesis

D81.1

Severe combined immunodeficiency [SCID] with low T- and B-cell numbers

D81.2

Severe combined immunodeficiency [SCID] with low or normal B-cell numbers

D81.3

Adenosine deaminase deficiency

D81.6

Major histocompatibility complex class I deficiency

D81.7

Major histocompatibility complex class II deficiency

D81.89

Other combined immunodeficiencies

D81.9

Combined immunodeficiency, unspecified

D82.0

Wiskott-Aldrich syndrome

D82.3

Immunodeficiency following hereditary defective response to Epstein-Barr virus

D82.4

Hyperimmunoglobulin E (IgE) syndrome

D82.8

Immunodeficiency associated with other specified major defects

D82.9

Immunodeficiency associated with major defect, unspecified

D83.0

Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function

D83.1

Common variable immunodeficiency with prominent immunoregulatory T-cell disorder

D83.2

Common variable immunodeficiency with autoantibodies to B- or T-cells

D83.8

Other common variable immunodeficiencies

D83.9

Common variable immunodeficiency, unspecified

D84.8

Other specified immunodeficiencies

D84.9

Immunodeficiency unspecified

D89.810

Acute graft-versus-host disease

D89.811

Chronic graft-versus-host disease

D89.812

Acute on chronic graft-versus-host disease

D89.813

Graft-versus-host disease unspecified

D89.82

Autoimmune lymphoprolipherative syndrome

D89.89

Other specified disorders involving immune mechanisms, not elsewhere classified

D89.9

Disorder involving the immune system, unspecified

G25.82

Stiff-man syndrome

G35

Multiple sclerosis

G60.3

Idiopathic progressive neuropathy

G60.8

Other hereditary and idiopathic neuropathies

G60.9

Hereditary and idiopathic neuropathies, unspecified

G61.0

Guillian-Barre syndrome

G61.81

Chronic inflammatory demyelinating polyneuritis

G61.82

Multifocal motor neuropathy

G61.9

Inflammatory polyneuropathy, unspecified

G62.89

Other specified polyneuropathies

G70.00

Myasthenia gravis without (acute) exacerbation

G70.01

Myasthenia gravis with (acute) exacerbation

G70.80

Lambert-Eaton syndrome, unspecified

G70.81

Lambert-Eaton syndrome in disease classified elsewhere

G73.1

Lambert-Eaton syndrome in neoplastic disease

G73.3

Myasthenic syndromes in other diseases classified elsewhere

J20.5

Acute bronchitis due to RSV

L10.0

Pemphigus vulgaris

L10.1

Pemphigus vegetans

L10.2

Pemphigus foliaceous

L10.3

Brazilian pemphigus (fogo selvagem)

L10.4

Pemphigus erythematosus

L10.5

Drug-induced pemphigus

L10.81

Paraneoplastic pemphigus

L10.89

Other pemphigus

L10.9

Pemphigus, unspecified

L12.0

Bullous pemphigoid

L12.1

Cicatricial pemphigoid

L12.30

Acquired epidermolysis bullosa, unspecified

L12.31

Epidermolysis bullosa due to drug

L12.35

Other acquired epidermolysis bullosa

L12.8

Other pemphigoid

L12.9

Pemphigoid, unspecified

L13.8

Other specified bullous disorders

L13.9

Bulous disorders, unspecified

L14

Bullous disorders in diseases classified elsewhere

L51.1

Stevens-Johnson syndrome

L51.2

Toxic epidermal necrolysis

L51.3

Stevens-Johnson syndrome-toxic epidermal necrolysis overlap syndrome

M30.3

Mucocutaneous lymph node syndrome (Kawasaki)

M33.00 – M33.09

Juvenile dermatopolymyositis, organ involvement

M33.10 – M33.19

Other dermatopolymyositis, organ involvement

M33.20 – M33.29

Polymyositis, organ involvement

M33.90 – M33.99

Dermatopolymyositis, organ involvement unspecified

M36.0

Dermato(poly)myositis in neoplastic disease

O98.511 – O98.53

Other viral diseases complicating pregnancy

P00.2

Newborn affected by maternal infectious and parasitic diseases

P07.00 – P07.30

Disorders relating to short gestation and low birthweight code

P35.0

Congenital rubella syndrome

P35.1

Congenital cytomegalovirus infection

P35.8

Other congenital viral diseases

P35.9

Congenital viral disease, unspecified

P37.8

Other specified congenital infections and parasitic diseases

P37.9

Congenital infectious or parasitic disease, unspecified

P55.0 – P55.1
P55.8 – P55.9

Hemolytic disease or fetus or newborn due to isoimmunization

P61.0

Transient neonatal thrombocytopenia

P61.5

Transient neonatal neutropenia

Q81.0 – Q81.9

Epidermolysis bullosa, unspecified

Q82.8

Other specified congenital malformations of skin

Q82.9

Congenital malformation of skin, unspecified

T30.0, T30.4

Burn of unspecified body region, unspecified degree

T86.00 – T86.99

Complications of transplanted organs

Z20.4

Contact with or exposure to rubella

Z20.6

Contact with or exposure to HIV

Z20.820

Contact or exposure to varicella

Z20.828

Contact or exposure to other viral diseases

Z41.8

Prophylactic immunotherapy

Z48.210 – Z48.298

Encounter for aftercare following transplant

Z76.82

Awaiting organ transplant

Z94.81 – Z94.9

Organ or tissue replaced by transplant

REIMBURSEMENT INFORMATION:

Refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

PPO Blue Script: Prior authorization is required. Authorization forms may be obtained from the Medication Review Unit of the Healthcare Program Management division.

Medicare Advantage Products: The following National Coverage Determination (NCD) was reviewed on the last guideline revised date: Intravenous Immune Globulin for the Treatment of Autoimmune Mucotaneous Blistering Disease, (250.3) located at cms.gov. The following Local Coverage Determinations (LCDs) were reviewed on the last guideline revised date: Intravenous Immune Globulin (L34007) located at fcso.com.

Medicare Part D: Florida Blue has delegated to Prime Therapeutics authority to make coverage determinations for the Medicare Part D services referenced in this guideline.

DEFINITIONS:

Agammaglobulinemia: lack of antibodies.

Antibody: a protein substance developed in response to and interacting specifically with an antigen. This antigen-antibody reaction forms the basis of immunity.

Antigen: a substance that induces the formation of antibodies that interact specifically with it.

Dysgammaglobulinemia: deficiencies in one or more classes of immunoglobulins in the blood.

Hypogammaglobulinemia: not enough antibodies relapsing/remitting: coming and going, worsening then improving.

Immunodeficiency: a deficiency of immune response or a disorder characterized by deficient immune response.

Immunoglobulin: one of a family of closely related proteins capable of acting as antibodies; five classes are IgG, IgA, IgM, IgD, and IgE.

Immunomodulator: an agent that specifically or nonspecifically augments or diminishes immune response, i.e., an adjuvant, immunostimulant or immunosuppressant.

Isohemagglutinin: a hemagglutinin that agglutinates the erythrocytes of other individuals of the same species.

Isoimmunization: the development of specific antibodies as a result of antigenic stimulation using material derived from the red blood cells of another individual.

Kawasaki Disease: a syndrome of unknown etiology, usually affecting infants and young children, associated with vasculitis of the large common vessels and numerous other systemic signs.

NEMO Syndrome: Nuclear factor kappa-B essential modulator (NEMO) deficiency results from mutations in the inhibitor of kappa-B kinase gamma chain gene. Disease characteristics may include immunodeficiency, ectodermal dysplasia and abnormal thermal regulation.

Specific Antibody Disorder: an immune disease in which children and adults fail to develop the immune response to the polysaccharide coating on bacteria but who otherwise have normal antibody levels.

WHIM Syndrome: Warts, hypogammaglobulinemia, immunodeficiency, and myelokathexis (WHIM) syndrome is a rare congenital immunodeficiency characterized by susceptibility to papilloma viruses, lymphocytopenia with decreased memory B-cell counts, hypogammaglobulinemia, and peripheral neutropenia with retention of mature neutrophils in the bone marrow.

RELATED GUIDELINES:

None applicable.

OTHER:

Documentation of medical necessity should include the following:

1. Care Provider Notes

2. All Laboratories Studies.

REFERENCES:

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COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Pharmacy Policy Committee on 07/13/16.

GUIDELINE UPDATE INFORMATION:

12/15/99

Medical Coverage Guideline Reformatted.

01/01/01

Annual HCPCS coding update.

07/15/01

3rd quarter HCPCS coding update.

12/15/02

Revision; consisting of updating coding.

01/01/06

HCPCS update, deleted expired codes J1563 and J1564, added new codes J1566 and J1567.

05/15/06

Review; added subcutaneous immune globulin.

01/01/07

HCPCS update, added J1562. MCG revised to include Medicare Part D as a program exception.

04/15/07

Review and revision; consisting of changing lab values for CVID for what is considered deficient, added note for CVID regarding normal IVIg but failure to produce antibodies with 2 consecutive pneumococcal or tetanus vaccines, added tables of IVIg laboratory values under OTHER, reformatted and updated references.

06/15/07

Revision; consisting of reformatting guideline; added HCPCS codes, modified criteria for agammaglobulinemia and updated references.

01/01/08

Annual coding update. Added CPT-4 code 90284, HCPCS codes J1561, J1568, J1569, J1571, J1572, J1573 and J2791. Deleted HCPCS codes J1567, Q4087, Q4088, Q4090, Q4091 and Q4092.

04/01/08

2nd Quarter HCPCS coding update (added Q4097).

04/15/08

Review and revision; consisting of renaming MCG, added 2 new indications, reformatted and updated references and links.

01/01/09

Annual HCPCS coding update: revised descriptor for code J1572; deleted codes Q4097, 90765 and 90766; added 96365, 96366, and J1459.

07/15/09

Review and revision; consisting of updating references.

06/15/10

Revision; consisting of adding new agent.

09/15/10

Review and revision; consisting of updating references and review of current literature.

10/01/10

Revision; consisting of removing criteria for MMN and updating references.

01/01/11

Revision; consisting of updating coding.

05/15/11

Revision; consisting of further defining indications and reformatting the position statement.

09/15/11

Review and revision to guideline; consisting of no changes to the position statement.

11/15/11

Revision to guideline; consisting of refining coverage criteria for functional immunodeficiency and updating coding.

01/01/12

Revision to guideline; consisting of updating coding.

09/15/12

Review and revision to guideline; consisting of updating position statement, precautions, coding and references.

12/15/12

Revision to guideline; consisting of updating coding.

03/15/13

Revision to guideline; consisting of updating position statement to include continuation criteria and adding new intravenous product.

05/15/13

Revision; Program Exceptions section updated.

08/15/13

Review and revision to guideline; consisting of revising position statement and updating references.

8/15/14

Review and revision to guideline; consisting of revising position statement and updating references.

01/01/15

Revision to guideline; consisting of update to Position Statement, Billing/Coding Information,

03/15/15

Revision to guideline; consisting of updating description and position statement.

08/15/15

Review and revision to guideline; consisting of revising position statement, warnings/precautions, coding and references.

09/15/15

Revision to guideline; consisting of updating coding.

10/01/15

Revision consisting of update to Program Exceptions section.

11/01/15

Revision: ICD-9 Codes deleted.

01/01/16

Annual HCPCS coding update: added code J1575.

08/15/16

Review and revision to guideline; consisting of revising description, position statement, dosing, warnings/precautions, coding and references.

09/15/16

Revision to site of service statement.

10/01/16

Update to ICD-10 codes.

10/15/16

Revision to site of service statement.

11/15/16

Revision to guideline; consisting of updating description and site of service statement with a new formulation.

Date Printed: June 26, 2017: 01:14 AM