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• Laboratory evidence of immunoglobulin deficiency may include the following definitions:
  • Agammaglobulinemia (total IgG less than 200 mg/dL)
  • Persistent hypogammaglobulinemia (total IgG less than 400 mg/dL, or at least two standard deviations below normal, on at least two occasions)
  • Absence of B lymphocytes
• Inability to mount an adequate antibody response to inciting antigens may include the following definitions:
  • Lack of appropriate rise in antibody titer following provocation with a polysaccharide antigen. For example, an adequate response to the pneumococcal vaccine may be defined as at least a four-fold increase in titers for at least 50% of serotypes tested.
  • Lack of appropriate rise in antibody titer following provocation with a protein antigen. For example, an adequate response to tetanus/diphtheria vaccine may be defined as less than a four-fold rise in titers 3-4 weeks after vaccine administration.

According to a 2010 national guideline from Canada on immune globulin for primary immune deficiency, although higher trough levels of IVIg may be associated with clinical response; the goal of IVIg dose increases should be to improve clinical effectiveness and not merely to increase trough levels.

Acute, severe ITP may be defined by the following parameters:

• acute ITP with major bleeding, e.g., life-threatening bleeding and/or clinically important mucocutaneous bleeding
• acute ITP with severe thrombocytopenia and at high risk for bleeding complications
• acute ITP with severe thrombocytopenia and a slow or inadequate response to corticosteroids
• acute ITP with severe thrombocytopenia and a predictable risk of bleeding in the future, e.g., a procedure or surgery with a high bleeding risk.

Patients with chronic inflammatory demyelinating neuropathy (CIDP) should meet the diagnostic criteria established by the American Academy of Neurology, particularly if the patient also is diagnosed with chronic fatigue syndrome. (See Appendix A for the diagnostic criteria.) In addition, by intravenous immunoglobulin infusion (IVIg), treatment should be limited to CIDP patients who do not respond to initial therapy with prednisone and are experiencing serious clinical worsening. In patients treated for chronic diseases, such as CIDP, multifocal motor neuropathy, and dermatomyositis, the effect of IVIg is transitory and therefore periodic infusions of IVIg are needed to maintain treatment effect. The frequency of transfusions is titrated to the treatment response; typically, biweekly or monthly infusions are needed. 

Investigative service is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized by certifying boards and/or approving or licensing agencies or published peer review criteria as standard, effective medical practice for the treatment of the condition being treated and as such therefore is not considered medically necessary.

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

8/1999: Revisions approved by MPAC

11/1999: Revisions approved by Pharmacy & Therapeutics (P & T) Committee

5/2001: Reviewed by MPAC; IgIV considered medically necessary for CIDP

1/30/2002: Venoglobulin I® deleted from ITP; Venoglobulin S®, Gammagond SD, Polygam SD added to Kawasaki syndrome

2/15/2002: Investigational definition added

5/1/2002: Type of Service and Place of Service deleted

5/29/2002: Code Reference section updated

11/6/2002: Prior authorization required

3/25/2003: Policy section revised, "CLL with frequent infections" deleted, CPT code range 90281-90399 deleted

7/2003: Reviewed by MPAC, Policy section aligned with BCBSA, treatment dosage information deleted, ICD-9 diagnosis code ranges 356.0-356.9, 493.00-493.91, 203.00-203.81, 714.0-714.9 listed separately, ICD-9 diagnosis code 695.4 deleted covered codes, ICD-9 diagnosis code 203.00-203.81, 288.0, 493.00-493.91 moved to non-covered codes, ICD-9 diagnosis code 493.92 added non-covered codes

10/13/2004: Code Reference section updated, CPT code 90780, 90781 deleted covered codes, ICD-9 diagnosis code 279.00, 279.04, 279.05, 279.12, 279.2, 357.82, 358.01, 776.1, V42.0, V42.1, V42.6, V42.7, V42.83 added covered codes, ICD-9 diagnosis code 357.0, 446.1 description revised, ICD-9 diagnosis 358.0 5th digit added, HCPCS J0850 added covered codes, HCPCS J1561 effective deletion date added, HCPCS J1562 deleted, non-covered ICD-9 diagnosis code 203.00-203.81, 288.0, 493.00-493.91, 493.92, 710.0, 714.0-714.9 deleted

11/11/05: Policy description revised: Intravenous immune globulin (IgIV) changed to (IVIg). Policy section language revised: Preferred provider changed to Accredo; telephone # changed from 1-866-591-9075 to 1-866-240-3373; fax # changed from 1-866-591-9094 to 1-800-711-3526. Policy section description revised for intravenous immune globulin therapy diagnoses related to medical necessity.  Added paragraph for intravenous immune globulin therapy.  Deleted under intravenous immune gloublin therapy: agammaglobulinemia - primary humoral, hypogammaglobulinemia - primary humoral immundeficiency, bacterial infections associated with B-Cell chronic lymphocytic leukemia (CLL), HIV-ITP with severe bleeding using pooled nonspecific IVIG preparations, prevention of infection in HIV-infected children(Gamimune N® only), solid organ transplant recipients at risk for cytomegalovirus infections and pneumonia (Cytogam® only). Vital capacity less than 1L, dysphagia associated with aspiration and inability to ambulate 100 feet without assistance deleted from definition of myasthenia gravis.  Deleted hereditary and idiopathic peripheral neuropathy, bone marrow transplant patients; and relapsing/remitting multiple sclerosis. Policy section description revised for polymyositis under the intravenous immune globulin therapy section related to investigational diagnoses;  added chronic progressive multiple sclerosis. Code Reference section updated: 5th digit added to ICD9 diagnosis code 287.31, description revised; HCPCS codes J1564, Q9941-Q9944 added, J1561 deleted.

11/2005: Approved by Pharmacy & Therapeutics (P & T) Committee

3/14/2006: Coding updated.  HCPCS 2006 revisions added to policy

3/20/2006: Policy reviewed, no changes

7/27/2006: Policy revised. Revisions approved by Medical Policy Advisory Committee (MPAC)

6/14/2007: Code Reference section updated per quarterly HCPCS and Category III revisions

12/19/2007: Coding updated per 2008 CPT/HCPCS revisions

1/18/2008: Policy revised to add "Policy" section on SCIg and maternal-fetal tolerance

1/21/2008: Code reference section revised; CPT 90284 and HCPCS J1562 added to covered codes. Added non-covered codes table; CPT 86021, 86355, 86357, 86360, 86361, and 86849 added to non-covered codes for techniques to investigate immunologic abnormalities affecting maternal-fetal tolerance. Policy named changed from "Intravenous Immune Globulin (IVIg)" to "Immune Globulin Replacement Therapy".

9/22/2008: Annual ICD-9 updates effective 10-1-2008 applied

1/1/2009:Accredo preferred provider information removed. BCBSMS information added.

1/6/2009: Policy reviewed, the following specifically listed under investigational:

• Fisher syndrome
• Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)
• Autism

 4/23/2009: ICD-9 code 340 deleted from covered table

10/11/2010: Updated the Code Reference section to remove 86360 and 86361 from the non-covered codes table.

02/28/1011:  Added new HCPCS codes J1559 and J1599 to the Code Reference section.

01/17/2012:  Policy statement reformatted for clarity purposes.  Add the following as medically necessary indications: Acute Humoral Rejection, Autoimmune Mucocutaneous Blistering Diseases, warm antibody autoimmune hemolytic anemia, refractory to corticosteroids and immunosuppressive agents, anti-phospholipid syndrome, and toxic shock syndrome.  Added the following indications to the investigational policy statement:  complex regional pain syndrome, Alzheimer’s disease, IGG sub-class deficiency, and sepsis.  Deleted the statement regarding laboratory tests to investigate immunologic abnormalities affecting maternal-fetal tolerance.  Policy guidelines updated regarding laboratory testing. Deleted the Non-Covered codes table. Deleted outdated references from the Sources section.

04/09/2013: Policy reviewed. Added neonatal sepsis and Crohn's disease as investigational indications. Removed deleted codes J1567 and Q4087 - Q4092 from the Code Reference section.

The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document. 

Covered Codes

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