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Date Printed: December 16, 2017: 09:26 PM

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09-J0000-59

Original Effective Date: 08/15/06

Reviewed: 08/09/17

Revised: 10/15/17

Subject: Rituximab (Rituxan®) and Rituximab;hyaluronidase (Rituxan Hycela™)

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.

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

DESCRIPTION:

Rituximab (Rituxan®) is a chimeric monoclonal antibody that targets CD20, which is primarily located on the surface of immune system B cells. Once rituximab binds to CD20, B-cells are destroyed; consequently, rituximab is used to treat diseases that are characterized by excessive amounts of B cells, by overactive B cells, or by dysfunctional B cells. Examples include lymphomas, leukemias, autoimmune disorders and in transplant rejection.

Rituximab was initially approved by the US Food and Drug Administration (FDA) in November 1997 for the treatment of relapsed or refractory B-cell Non-Hodgkin’s Lymphoma (NHL). Additional FDA-labeled indications include Chronic Lymphocytic Leukemia (CLL), rheumatoid arthritis (RA), and granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis)/microscopic polyangiitis (MPA). Standard reference compendia (e.g., Clinical Pharmacology, DrugDex) support rituximab use in a plethora of off-label indications include autoimmune blistering diseases, autoimmune hemolytic anemia, neuromyelitis optica, and many more.

Rituximab;hyaluronidase (Rituxan Hycela™) was FDA approved in June 2017 for adult patients with follicular lymphoma, diffuse large B-cell lymphoma, and chronic lymphocytic leukemia. Rituximab;hyaluronidase is administered subcutaneously and is only recommended following an initial dose of intravenous rituximab. It is not indicated for the treatment of non-malignant conditions. Hyaluronidase increases the permeability of subcutaneous tissue by temporarily depolymerizing hyaluronan, a polysaccharide in subcutaneous tissue. This results in dispersion and absorption of rituximab when administered subcutaneously in combination with hyaluronidase. Rituximab;hyaluronidase has demonstrated non-inferior trough concentrations and comparable efficacy and safety to intravenous rituximab.

POSITION STATEMENT:

 

Certificate of Medical Necessity

Submit a completed Certificate of Medical Necessity (CMN) along with your request to expedite the medical review process.

1. Click the link Rituximab (Rituxan) - Certificate of Medical Necessity (MS Word) to open the form.

2. Complete all fields on the form thoroughly.

3. Print and submit a copy of the form with your request.

Note: Florida Blue regularly updates CMNs. Ensure you are using the most current copy of a CMN before submitting to Florida Blue. For a complete list of available CMNs, visit the Certificates of Medical Necessity page.

1. Initiation of rituximab (Rituxan®) meets the definition of medical necessity when administered for the indications listed in Table 1 and ALL of the indication-specific and maximum-allowable dose criteria are met:

Table 1

Indications and Specific Criteria

Indication

Specific Criteria

Maximum Allowable Dose

Non-oncology related

Autoimmune blistering disease (ANY of the following)

• Pemphigus vulgaris

• Pemphigus foliaceus

• Bullous pemphigoid

• Cicatricial pemphigoid

• Epidermolysis bullosa acquisita

• Paraneoplastic pemphigus

When BOTH of the following are met:

1. Member had an inadequate response to corticosteroids

2. Member had an inadequate response to oral immunosuppressant therapy including ONE or more of the following:

a. Mycophenolate

b. Azathioprine

c. Cyclosporine

d. Methotrexate

375 mg/m2 per dose*

Autoimmune hemolytic anemia, cold type (AIHA)

Diagnosis only

375 mg/m2 per dose

AIHA, warm type

When the member had an inadequate response to a trial of corticosteroids (e.g., prednisone 1 mg/kg/day for 3 weeks)

375 mg/m2 per dose

Castleman’s Disease

Member meets ONE of the following:

1. Diagnosis of Multicentric Castleman’s Disease

2. Unicentric Castleman’s Disease and ONE of the following is met:

a. Disease is surgically unresectable

b. Member is symptomatic following partial resection

c. Disease is relapsed or refractory

375 mg/m2 per dose

Evan’s syndrome

Documented inadequate response, contraindication, or intolerance to conventional therapy (e.g.,corticosteroids, azathioprine, cyclophosphamide, cyclosporine)

375 mg/m2 per dose

Graft versus host disease, chronic (GVHD)

When BOTH of the following are met:

1. Member had an inadequate response to corticosteroids

2. Member had an inadequate response to immunosuppressants (e.g., cyclosporine, tacrolimus, sirolimus)

375 mg/m2 per dose

Heart transplant

When the member is receiving therapy for ONE of the following:

1. Desensitization for highly-allosensitized transplant candidates

2. Anti-body mediated rejection (AMR)

375 mg/m2 per dose

Hemophilia, acquired factor inhibitors

When the member has an inadequate response or contraindication to ONE of the following:

1. Adequate trial of corticosteroids or cyclophosphamide (e.g., 4-6 weeks)

2. Immune tolerance induction therapy

375 mg/m2 per dose

Idiopathic or immune thrombocytopenic purpura, chronic (ITP)

When the member is at risk of bleeding and BOTH of the following are met:

1. The member has demonstrated an inadequate response to ANY of the following:

a. Adequate trial of corticosteroids (e.g., prednisone 1-2 mg/kg for 2-4 weeks)

b. IVIG

c. Splenectomy

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

375 mg/m2 per dose

Myasthenia Gravis, refractory

When the member has progressive disease with an inadequate response, contraindication, or intolerance to ALL of the following:

1. pyridostigmine

2. corticosteroids

3. azathioprine

4. cyclosporine

5. IVIG

375 mg/m2 per dose

Neuromyelitis optica (NMO)

When the member had an inadequate response to a trial (e.g., 3 months) of one or more of the following oral immunosuppressants

1. Azathioprine

2. Methotrexate

3. Mycophenolate

1000 mg administered on day 1 and 15 every 24 weeks

Pediatric idiopathic nephrotic syndrome

When BOTH of the following are met:

1. Member’s disease is dependent on or refractory to corticosteroids

2. Member had an inadequate response to oral immunosuppressant therapy (e.g., tacrolimus, cyclosporine, mycophenolate)

375 mg/m2 per dose

Renal Transplant

When used to prevent graft rejection in members with anti-donor antibodies (e.g., anti-HLA antibodies)

375 mg/m2 per dose

Rheumatoid Arthritis (RA)

When ALL of the following are met:

1. Member’s disease is moderately to severely active

2. Rituximab will be used in combination with methotrexate (unless member has a contraindication to methotrexate)

3. Member is 18 years of age or older

4. Member has at least ONE of the following:

A. Member has an inadequate response to atrial (e.g., 3 months) of one or more of the following biologic therapies:

a. Adalimumab (Humira)

b. Abatacept (Orencia)

c. Certolizumab Pegol (Cimzia)

d. Etanercept (Enbrel)

e. Golimumab (Simponi, Simponi Aria)

f. Infliximab (Remicade)

g. Tocilizumab (Actemra)

B. Member has a history of treated lymphoproliferative malignancy

1000 mg administered on day 1 and 15 every 16 weeks

Systemic autoimmune diseases

When the member is diagnosed with ONE of the following:

1. Cryoglobulinemia

2. Primary Sjögren Syndrome

375 mg/m2 per dose

Systemic Lupus Erythematosus

When the member has ONE of the following:

1. The member has an inadequate response or has a contraindication to at least 2 immunosuppressants (e.g., mycophenolate mofetil, cyclophosphamide, azathioprine)

2. The member has nephritis that has an inadequate response to a trial of immunosuppressant therapy (e.g., 6 months)

375 mg/m2 per dose

Thrombotic thrombocytopenic purpura (TTP)

When the member has an inadequate response to plasma exchange and corticosteroids

375 mg/m2 per dose

Vasculitides

• Granulomatosis with Polyangiitis (GPA or Wegener’s granulomatosis)

• Churg-Strauss syndrome

• Microscopic polyangiitis (MPA)

• Pauci-immune glomerulonephritis

When rituximab will be used in combination with corticosteroids

375 mg/m2 per dose

Oncology-related

Acute lymphoblastic leukemia (ALL)

When the member has CD20 positive disease and ONE of the following are met:

1. ALL is Philadelphia chromosome negative (Ph-) and rituximab is used with ONE of the following:

a. GRAALL-2005 regimen (daunorubicin, vincristine, prednisone, pegaspargase, and cyclophosphamide)

b. HyperCVAD (hyper-fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate and cytarabine

c. Idarubicin, dexamethasone, vincristine, cyclophosphamide and cytarabine

d. MOpAD regimen (methotrexate, vincristine, pegaspargase, dexamethasone)

2. ALL is Philadelphia chromosome positive (Ph+) and all of the following are met:

a. Member has relapsed or refractory disease

b. Member’s ALL is refractory to tyrosine kinase inhibitors

c. Use is in combination with MOpAD regimen (methotrexate, vincristine, pegaspargase, dexamethasone)

375 mg/m2 per dose

Central nervous system (CNS) Cancer-Leptomeningeal Metastases

Rituximab will be administered intrathecally in members who meet ONE of the following:

1. Used as induction therapy in good risk patients (KPS >60, no major neurologic deficits, minimal systemic disease) with normal CSF flow

2. Used as maintenance therapy for members with negative CSF cytology

3. Used as maintenance therapy for clinically stable members with positive CSF cytology

4. Used as postinduction therapy for members with positive CSF cytology

25 mg per dose

Central nervous system (CNS) Cancers – Primary CNS Lymphoma

When ONE of the following is met:

1. For induction therapy in combination with EITHER:

  • High-dose methotrexate, vincristine, procarbazine and cytarabine
  • High-dose methotrexate with or without temozolomide

2. For progressive/recurrent disease when used as a single agent or in combination with temozolomide in members who meet ONE of the following:

  • Member has received prior whole brain radiation
  • Member has received prior methotrexate-based regimen without prior radiation
  • Member received prior high-dose chemotherapy with stem cell rescue with a previous response of >12 months

500 mg/m2 per dose

Chronic lymphocytic Leukemia (CLL)/ Small Lymphocytic Lymphoma (SLL)

When the member’s disease is CD20-positive

500 mg/m2 per dose

Hodgkin Lymphoma

375 mg/m2 per dose

Non-Hodgkin Lymphoma

375 mg/m2 per dose

Post-transplant lymphoproliferative disease (PTLD)

When used for ONE of the following:

1. first-line therapy for monomorphic or polymorphic PTLD

2. second-line therapy for persistent or progressive early lesions

3. second-line therapy for persistent or progressive monomorphic or polymorphic PTLD

4. maintenance therapy for polymorphic PTLD achieving complete response on first-line therapy

5. member’s disease is refractory to a reduction in immunosuppression

375 mg/m2 per dose

Waldenström’s macroglobulinemia/Lymphoplasmacytic lymphoma

Diagnosis only

375 mg/m2 per dose

*Alternative dose: 1000 mg given on day 1 and 15

Approval duration: 6 months for all indications except RA (approval duration is 16 weeks).

2. Continuation of rituximab meets the definition of medical necessity for the indications in Table 1 when ALL of the following criteria are met:

a. Member has a history of beneficial response to therapy

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

c. The dose does not exceed ANY of the following based on indication for therapy:

i. Neuromyelitis optica: 1000 mg administered on day 1 and 15 every 24 weeks

ii. Rheumatoid arthritis: 1000 mg administered on day 1 and 15 every 16 weeks

iii. Chronic lymphocytic leukemia,Small lymphocytic lymphoma, or Primary CNS Lymphoma: 500 mg/m2 per dose

iv. CNS cancer, Leptomeningeal Metastases (Intrathecal administration): 25 mg per dose

v. All other indications: 375 mg/m2 per dose

Approval duration: 1 year

1. Initiation of rituximab hyaluronidase (Rituxan Hycela™) meets the definition of medical necessity when administered following an initial single dose of IV rituximab for the indications listed in Table 2 and ALL of the indication-specific and maximum-allowable dose criteria are met:

Table 2

Indications and Specific Criteria

Indication

Specific Criteria

Maximum Allowable Dose

AIDS-related B-cell Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Burkitt Lymphoma

When used as a substitute for rituximab with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Castleman’s Disease (CD)

When used as a substitute for rituximab as a single agent or with other systemic therapies and member has ONE of the following:

1. Multicentric CD

2. Unicentric CD and ONE of the following is met:

a. Disease is surgically unresectable

b. Member is symptomatic following partial resection

c. Disease is relapsed or refractory

1400 mg per dose

Chronic Lymphocytic Leukemia (CLL)/ Small Lymphocytic Lymphoma (SLL)

When used in combination with fludarabine and cyclophosphamide (FC)

1600 mg per dose

Diffuse Large B-cell Lymphoma (DLBCL)

ONE of the following is met:

1. When BOTH of the following are met:

a. Member has previously untreated diffuse large B-cell lymphoma

b. Use is in combination with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) or other anthracycline-based chemotherapy regimens

2. When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Follicular Lymphoma (FL)

ONE of the following is met:

1. Use is in combination with first line chemotherapy in previously untreated follicular lymphoma

2. Single agent use for ONE of the following:

a. Relapsed or refractory, follicular lymphoma

b. Maintenance therapy in members achieving a complete or partial response to rituximab in combination with chemotherapy

c. Non-progressing (including stable disease), follicular lymphoma after first-line cyclophosphamide, vincristine, and prednisone (CVP) chemotherapy

3. When used as a substitute for rituximab as a single agent or with other systemic therapies† and member’s disease is CD20-positive

1400 mg per dose

Gastric MALT Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Mantle Cell Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Nodal Marginal Zone Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Nongastric MALT Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Post-transplant Lymphoproliferative Disorder (PTLD)

When used as a substitute for rituximab as a single agent or with other systemic therapies for ONE of the following:

1. first-line therapy for monomorphic or polymorphic PTLD

2. second-line therapy for persistent or progressive early lesions

3. second-line therapy for persistent or progressive monomorphic or polymorphic PTLD

4. maintenance therapy for polymorphic PTLD achieving complete response on first-line therapy

1400 mg per dose

Splenic Marginal Zone Lymphoma

When used as a substitute for rituximab as a single agent or with other systemic therapies and member’s disease is CD20-positive

1400 mg per dose

Approval duration: 6 months

2. Continuation of rituximab hyaluronidase (Rituxan Hycela™) meets the definition of medical necessity for the indications in Table 2 when ALL of the following criteria are met:

a. Member has a history of beneficial response to therapy

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

c. The dose does not exceed indication specific dosing in Table 2.

Approval duration: 1 year

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.

Rituximab

FDA-approved: rituximab is indicated for treatment of non-Hodgkin’s Lymphoma (NHL), chronic lymphocytic leukemia (CLL), rheumatoid arthritis (RA), and granulomatosis with polyangiitis (GPA) (Wegener’s Granulomatosis) and microscopic polyangiitis (MPA). Rituximab should be used in combination with methotrexate in individuals with moderately to severely active RA who have had an inadequate response to one or more TNF antagonist therapies. The recommended dosage regimens for FDA-approved indications are listed in table 3.

Table 3

FDA-approved indications and dosage regimens

Indication

Dose

NHL

375 mg/m2

CLL

375 mg/m2 prior to initiation of FC, then 500 mg/m2 on day1 of cycles 2-6

RA

Two-1000 mg infusions separated by 2 weeks; subsequent infusions should administered every 24 weeks or based on clinical evaluation. Do not administer sooner than every 16 weeks

GPA/MPA

375 mg/m2 once weekly for 4 weeks±

Schedule is based on type of NHL, dose is 250 mg/m2 if used as a component of Zevalin® for NHL

Administer methylprednisolone 100 mg IV or its equivalent 30 minutes prior to each infusion

± Administration of methylprednisolone 1000 mg IV daily for 1-3 days followed by oral prednisone 1 mg/kg/day (not to exceed 80 mg/day and tapered per clinical need) are recommended to treat severe vasculitis symptoms.

NHL, non-Hodgkin’s Lymphoma; CLL, chronic lymphocytic leukemia; FC, fludarabine and cyclophosphamide; RA, rheumatoid arthritis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.

Rituximab should be administered as an intravenous (IV) infusion and should not be administered as an IV push or IV bolus. Premedication should be administered prior to each infusion (e.g., acetaminophen, antihistamine). Initially, rituximab should be administered at a rate of 50 mg/hr; in the absence of infusion toxicity, the rate can be increased by 50 mg/hr increments at 30-minute intervals to a maximum of 400 mg/hr.

Rituximab; Hyaluronidase

FDA-approved: rituximab; hyaluronidase is indicated for treatment of Follicular Lymphoma (FL), Diffuse Large B-cell Lymphoma (DLBCL), and Chronic Lymphocytic Leukemia (CLL), Rituximab;hyaluronidase is not indicated for the treatment of non-malignant conditions.

All patients must receive at least one full dose of a rituximab product by intravenous infusion before receiving rituximab;hyaluronidase by subcutaneous injection. Rituximab;hyaluronidase should be administered by a healthcare professional with medical support to manage severe reactions. Premedicate with acetaminophen and antihistamine before each dose and consider premedication with glucocorticoids.

FL/DLBCL: Administer 1,400 mg/23,400 Units (1,400 mg rituximab and 23,400 Units hyaluronidase human) subcutaneously. See prescribing information for recommended schedule and use of concomitant medications.

CLL: Administer 1,600 mg/26,800 Units (1,600 mg rituximab and 26,800 Units hyaluronidase human) subcutaneously. See prescribing information for recommended schedule and use of concomitant medications.

Administer specified volume into subcutaneous tissue of abdomen and observe 15 minutes following administration. Do not administer other subcutaneous medications at the same site of administration.

11.7 mL from 1,400 mg/23,400 Units vial over approximately 5 minutes.

13.4 mL from 1,600 mg/26,800 Units vial over approximately 7 minutes.

Drug Availability:

Rituximab is supplied as a 100 mg/10 ml and 500 mg/50 mL solution in a single-use vial.

Rituximab;hyaluronidase is supplied as 1,400 mg/23,400 units per 11.7 mL and 1,600 mg/26,800 units per 13.4 mL

PRECAUTIONS:

Boxed Warning

• Fatal infusion reactions within 24 hours of rituximab infusion can occur; approximately 80% of fatal reactions occurred with first infusion. Monitor members and discontinue rituximab infusion for severe reactions

• Severe mucocutaneous reactions, some with fatal outcomes can occur.

• Hepatitis B reactivation with fulminant hepatitis, hepatic failure and death; screen patients for HBV prior to initiation and monitor patients during and several months after therapy. Discontinue rituximab if reactivation occurs

• Progressive multifocal leukoencephalopathy (PML) resulting in death can occur in persons receiving rituximab. Monitor neurologic function and discontinue rituximab if PML occurs.

Warnings

• Tumor lysis syndrome (TLS): acute renal failure, hyperkalemia, hypocalcemia, hyperuricemia or hyperphosphatemia, requiring dialysis with instances of fatal outcome can occur in the setting of TLS following treatment of NHL persons with rituximab. Administer aggressive IV hydration, anti-hyperuricemic agents, and monitor renal function

• Infections – serious, fatal infections can occur. Withhold rituximab and institute appropriate anti-infective therapy

• Cardiac arrhythmias and angina can occur and can be life threatening. Monitor individuals with these conditions closely

• Renal toxicity – severe, fatal renal toxicity can occur. Monitor for signs and symptoms of renal failure.

• Bowel obstruction and perforation – some cases have lead to death, evaluate complaints of abdominal pain

• Do not administer live virus vaccines prior to or during rituximab

• Monitor CBC and platelet counts prior to therapy and at regular intervals for severe cytopenias

• Hypersensitivity reactions and local cutaneous reactions may occur during administration or more than 24 hours after subcutaneous administration. Premedicate and Interrupt if severe infusion reaction occurs.

• Embryo-fetal toxicity- may cause harm to a developing fetus and effective contraception should be utilized up to 12 months following administration.

BILLING/CODING INFORMATION:

HCPCS Coding for rituximab:

J9310

Rituximab, 100mg

HCPCS Coding for rituximab; hyaluronidase:

C9399

Unclassified drugs or biologicals (hospital outpatient use only)

J9999

Not otherwise classified, antineoplastic drugs

ICD-10 Diagnosis Codes That Support Medical Necessity for rituximab (J9310):

B10.89

Other human herpes virus infection

B20

Human immunodeficiency virus [HIV] disease

C79.32

Secondary malignant neoplasm of cerebral meninges

C79.40

Secondary malignant neoplasm of unspecified part of nervous system

C79.49

Secondary malignant neoplasm of other parts of nervous system

C81.00 – C81.09

Nodular lymphocyte predominant Hodgkin lymphoma

C81.40 – C81.49

Lymphocyte-rich Hodgkin lymphoma

C82.00 – C82.69

Follicular lymphoma of various sites

C82.80 – C82.99

Other specified types of follicular lymphoma and unspecified follicular lymphoma

C83.00 – C83.99

Small cell B-cell lymphoma, Mantle cell lymphoma, diffuse large B-cell lymphoma, lymphoblastic (diffuse) lymphoma, Burkitt lymphoma and other non-follicular lymphoma

C84.90 – C84.99

Cutaneous T-cell lymphoma various sites

C84.A0 – C84.A9

Other mature T/NK-cell lymphomas, various sites

C84.Z0 – C84.Z9

Mature T/NK-cell lymphomas, unspecified various sites

C85.10 – C85.99

Other specified and unspecified types of non-Hodgkin lymphoma, various sites

C86.0 – C86.66

Other specified types of T/NK-cell lymphoma

C88.0

Waldenstrom macroglobulinemia

C88.4

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (Malt- lymphoma)

C91.00

Acute lymphoblastic leukemia not having achieved remission

C91.01

Acute lymphoblastic leukemia, in remission

C91.02

Acute lymphoblastic leukemia, in relapse

C91.10

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

C91.12

Chronic lymphocytic leukemia of B-cell type in relapse

C91.40 – C91.42

Hairy cell leukemia

C91.90

Lymphoid leukemia, unspecified not having achieved remission

D36.0

Benign neoplasm of lymph nodes

D47.Z1

Post-transplant lymphoproliferative disorder (PTLD)

D47.Z2

Castleman disease

D59.0

Drug-induced autoimmune hemolytic anemia

D59.1

Other autoimmune hemolytic anemias

D66

Hereditary factor VIII deficiency

D67

Hereditary factor IX deficiency

D68.1

Hereditary factor XI deficiency

D68.311

Acquired hemophilia

D69.3 – D69.49

Immune thrombocytopenic purpura, Evans syndrome, congenital and hereditary thrombocytopenia purpura and other primary thrombocytopenia

D69.6

Thrombocytopenia, unspecified

D89.1

Cryoglobulinemia

D89.811 – D89.813

Chronic, graft-versus-host disease

G36.0

Neuromyelitis optica [Devic]

G70.00 – G70.01

Myasthenia gravis

L10.0 – L10.9

Pemphigus vulgaris and other pemphigus types

L12.0 – L12.9

Pemphigoid of various types

L13.8

Other specified bullous disorders

L13.9

Bullous disorder, unspecified

L14

Bullous disorders in diseases classified elsewhere

L51.2

Toxic epidermal necrolysis [Lyell]

M05.00 – M05.09

Felty's syndrome, various sites

M05.10 – M05.19

Rheumatoid lung disease with rheumatoid arthritis of various sites

M05.20 – M05.29

Rheumatoid vasculitis with rheumatoid arthritis of various sites

M05.30 – M05.39

Rheumatoid heart disease with rheumatoid arthritis of various sites

M05.40 – M05.49

Rheumatoid myopathy with rheumatoid arthritis of various sites

M05.50 – M05.59

Rheumatoid polyneuropathy with rheumatoid arthritis of various sites

M05.60 – M05.69

Rheumatoid arthritis with involvement of other organs and systems, various sites

M05.70 – M05.79

Rheumatoid arthritis with rheumatoid factor without involvement of other organs and systems, various sites

M05.80 – M05.89

Other rheumatoid arthritis with rheumatoid factor of various sites

M05.9

Rheumatoid arthritis with rheumatoid factor, unspecified

M06.00 – M06.09

Rheumatoid arthritis without rheumatoid factor, various sites

M06.1

Adult-onset Still's disease

M06.4

Inflammatory polyarthropathy

M06.80 – M06.89

Other specified rheumatoid arthritis, various sites

M06.9

Rheumatoid arthritis, unspecified

M30.0

Polyarteritis nodosa

M30.1

Polyarteritis with lung involvement [Churg-Strauss]

M31.0

Hypersensitivity angiitis

M31.1

Thrombotic microangiopathy

M31.30

Wegener's granulomatosis without renal involvement

M31.31

Wegener’s granulomatosis with renal involvement

M31.7

Microscopic polyangiitis

M32.0 – M32.9

Systemic lupus erythematosus, organ or system involvement unspecified

M35.00 – M35.09

Sicca syndrome, various areas of involvement

N04.0 – N04.9

Nephrotic syndrome

N05.0 – N05.9

Unspecified nephritic syndrome

Q81.0 – Q81.9

Epidermolysis bullosa, unspecified

Q82.9

Congenital malformation of skin, unspecified

Q82.8

Other specified congenital malformations of skin

R59.0 – R59.9

Enlarged lymph nodes, unspecified

T86.00 – T86.02

Complications of bone marrow transplant

T86.09

Other complications of bone marrow transplant

T86.10 – T86.12

Complications of kidney transplant

T86.19

Other complications of kidney transplant

T86.20 – T86.22

Complications of heart transplant

T86.298

Other complications of heart transplant

T86.30 – T86.32

Complications of heart-lung transplant

T86.39

Other complications of heart-lung transplant

T86.40 – T86.42

Complications of liver transplant

T86.49

Other complications of liver transplant

T86.5

Complications of stem cell transplant

T86.810 – T86.811

Complications of lung transplant

T86.818 – T86.819

Other or Unspecified complications of lung transplant

T86.820 – T86.821

Complications of skin graft (allograft) (autograft)

T86.828 – T86.829

Other or unspecified complications of skin graft (allograft) (autograft)

T86.830 – T86.831

Complications of bone graft

T86.838 – T86.839

Other or unspecified complication of bone graft

T86.840 – T86.841

Complications of corneal transplant

T86.848 – T86.849

Other or unspecified complication of corneal transplant

T86.850 – T86.851

Complication of intestine transplant

T86.858 – T86.859

Other or Unspecified complications of intestine transplant

T86.890 – T86.891

Complications of other transplanted tissue

T86.898 – T86.899

Other or Unspecified complication of other transplanted tissue

T86.90 – T86.92

Complication of unspecified transplanted organ and tissue

T86.99

Other complications of unspecified transplanted organ and tissue

Z48.22

Encounter for aftercare following kidney transplant

Z94.0

Kidney transplant status

ICD-10 Diagnosis Codes That Support Medical Necessity for rituximab;hyaluronidase (C9399, J9999):

B20

Human immunodeficiency virus [HIV] disease

C82.00 – 82.99

Follicular lymphoma of various sites

C83.00 – C83.09

Small cell B-cell lymphoma

C83.10 – C83.19

Mantle cell lymphoma

C83.30 – C83.39

Diffuse large B-cell lymphoma

C83.70 – C83.89

Burkitt lymphoma

C85.80 – C85.89

Other specified types of non-Hodgkin lymphoma

C88.4

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]

C91.10

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

C91.12

Chronic lymphocytic leukemia of B-cell type in relapse

D36.0

Benign neoplasm of lymph nodes

D47.Z1

Post-transplant lymphoproliferative disorder (PTLD)

R59.0 – R59.9

Enlarged lymph nodes

REIMBURSEMENT INFORMATION:

Please refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

Medicare Advantage products: No National Coverage Determination (NCD) was found at the time of the last guideline revised date. The following Local Coverage Determination (LCD) was reviewed on the last guideline revised date: Rituximab, (L33746) located at fcso.com.

DEFINITIONS:

RA: Rheumatoid Arthritis.

TNF: tumor necrosis factor.

HyperCVAD: hyper-fractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone

RELATED GUIDELINES:

Abatacept (Orencia®), 09-J0000-67

Adalimumab (Humira®), 09-J0000-46

Anakinra (Kineret®), 09-J0000-45

Certolizumab Pegol (Cimzia®), 09-J0000-77

Etanercept (Enbrel®), 09-J0000-38

Golimumab (Simponi™), 09-J1000-11

Infliximab (Remicade®), 09-J0000-39

OTHER:

None applicable.

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COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Pharmacy Policy Committee on 08/09/17.

GUIDELINE UPDATE INFORMATION:

08/15/06

New Medical Coverage Guideline.

10/15/06

Revised: added highlighted note stating that this MCG only addresses use in rheumatoid arthritis and does not address rituximab use in oncological applications, revised descriptor of HCPCS code and removed Medicare Advantage from program exceptions.

06/15/07

Review and revision; consisting of reformatting guideline, adding Remicade® in parentheses in criteria after failure of TNF antagonists, added ICD-9 codes in order to be compliant with FCSO LCD, updated related guidelines and updated references.

05/15/08

Review and revision; consisting of adding a black box warning under “PRECAUTIONS” and updating references.

11/15/08

Revision; consisting of updating the boxed warning under the Precautions section and removing the experimental and investigational statement from the Position Statement and adding “Re-treatment is not supported in the literature sooner than 6 months after initial treatment AND is investigational”.

01/01/09

Annual HCPCS coding update: revised descriptor for code J9310; deleted 90765 and 90766; added 96365 and 96366.

05/15/09

Revision; consisting of changing name of guideline, adding oncologic indications, maximum doses and updating references.

09/15/09

Review and revision; consisting of updating references.

10/15/09

Revision; consisting of clarifying dosage and update coding.

08/01/10

Revision; consisting of updating coding.

10/15/10

Revision; consisting of updating coding.

02/01/11

Review and revision; consisting of adding new indication, removing an indication, updating approved dosages, and references and coding.

05/15/11

Revision to guideline; consisting of updating coding and adding alternate dosing regimen.

07/15/11

Revision to guideline; consisting of add 2 new indications.

01/15/11

Review and revision to guideline; consisting of updating references and coding

07/15/12

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

12/15/12

Revision to guideline; consisting of updating coding.

04/15/13

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

09/15/13

Review and revision to guideline; consisting of revising and reformatting position statement, revising description section, dosage/administration section, precautions section, updated program exceptions and references.

09/15/14

Review and revision to guideline; consisting of reformatting the position statement; updating references and coding.

04/15/15

Revision to guidelines; updating coding.

09/15/15

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

10/01/15

Revision consisting of update to Program Exceptions section.

11/01/15

Revision: ICD-9 Codes deleted.

05/15/16

Revision to codes.

06/15/16

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

10/01/16

Update to ICD-10 codes.

09/15/17

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

10/15/17

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

Date Printed: December 16, 2017: 09:26 PM