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Multiple myeloma (MM) is a systemic malignancy of plasma cells that represents approximately 10% of all hematologic cancers. It is treatable but rarely curable. POEMS syndrome is a rare, paraneoplastic disorder secondary to a plasma-cell dyscrasia. The acronym POEMS reflects the hallmark characteristics of the syndrome: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes.
Hematopoietic Stem-Cell Transplantation
Immunologic compatibility between infused hematopoietic stem cells and the recipient is not an issue in autologous HSCT. However, immunologic compatibility between donor and patient is a critical factor for achieving a good outcome of allogeneic HSCT. Compatibility is established by typing of human leukocyte antigens (HLA) using cellular, serologic, or molecular techniques. HLA refers to the tissue type expressed at the Class I and Class II loci on each arm of chromosome 6. Depending on the disease being treated, an acceptable donor will match the patient at all or most of the HLA loci (with the exception of umbilical cord blood).
Conventional Preparative Conditioning for HSCT
The success of autologous HSCT is predicated on the ability of cytotoxic chemotherapy with or without radiation to eradicate cancerous cells from the blood and bone marrow. This permits subsequent engraftment and repopulation of bone marrow space with presumably normal hematopoietic stem cells obtained from the patient prior to undergoing bone marrow ablation. As a consequence, autologous HSCT is typically performed as consolidation therapy when the patient’s disease is in complete remission. Patients who undergo autologous HSCT are susceptible to chemotherapy-related toxicities and opportunistic infections prior to engraftment, but not GVHD.
Reduced-Intensity Conditioning for Allogeneic HSCT
For the purposes of this Policy, the term “reduced-intensity conditioning” will refer to all conditioning regimens intended to be non-myeloablative, as opposed to fully myeloablative (traditional) regimens.
The disease is staged by estimating tumor mass, based on various clinical parameters like hemoglobin, serum calcium, number of lytic bone lesions, and the presence or absence of renal failure. Multiple myeloma usually evolves from an asymptomatic premalignant stage (termed monoclonal gammopathy of undetermined significance). Treatment is usually reserved for patients with symptomatic disease (usually progressive myeloma), whereas asymptomatic patients are observed, as there is little evidence that early treatment of asymptomatic multiple myeloma prolongs survival when compared to therapy delivered at the time of symptoms or end-organ damage. In some patients, an intermediate asymptomatic but more advanced premalignant stage is recognized, and referred to as smoldering multiple myeloma. The overall risk of disease progression from smoldering to symptomatic multiple myeloma is 10% per year for the first 5 years, approximately 3% per year for the next 5 years, and 1% for the next 10 years.
POEMS syndrome (also known as osteosclerotic myeloma, Crow-Fukase syndrome, or Takasuki syndrome) is a rare, paraneoplastic disorder secondary to a plasma-cell dyscrasia. This complex, multiorgan disease was first described in 1938, but the acronym POEMS was coined in 1980, reflecting hallmark characteristics of the syndrome: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. No single test establishes the presence of POEMS syndrome. Its pathogenesis is undefined, although some evidence suggests it is mediated by imbalance of proinflammatory cytokines including interleukin (IL)-1β (IL-1β), IL-6, and tumor necrosis factor-α; vascular endothelial growth factor may also be involved. However, specific criteria have been established, and the syndrome may entail other findings in the constellation of signs and symptoms, as shown in the Table. Both major criteria and at least one of the minor criteria are necessary for diagnosis.
The prevalence of POEMS syndrome is unclear. A national survey in Japan showed a prevalence of about 0.3 per 100,000. Other large series have been described in the United States and in India. In general, patients with POEMS have a superior overall survival compared with that of MM, nearly 14 years in a large series from the Mayo Clinic. However, given the rarity of POEMS, no randomized controlled trials of therapies have been reported. Numerous approaches have included ionizing radiation, plasmapheresis, intravenous immunoglobulin, interferon alfa, corticosteroids, alkylating agents, azathioprine, tamoxifen, transretinoic acid, and high-dose chemotherapy with autologous HSCT support. Optimal treatment involves eliminating the plasma cell clone, for example, by surgical excision or local radiotherapy for an isolated plasmacytoma, or systemic chemotherapy in patients with disseminated disease, such as medullary disease or multiple plasmacytomas. Given the underlying plasma cell dyscrasia of POEMS, newer approaches to MM, including bortezomib, lenalidomide, and thalidomide, are also under investigation.
POLICYNo benefits will be provided for a covered transplant procedure unless the Member receives prior authorization through Case Management from Blue Cross & Blue Shield of Mississippi.
A single or second (salvage) autologous hematopoietic stem-cell transplantation may be considered medically necessary to treat multiple myeloma.
Tandem autologous-autologous hematopoietic stem-cell transplantation may be considered medically necessary to treat multiple myeloma in patients who fail to achieve at least a near-complete or very good partial response after the first transplant in the tandem sequence. (For definitions of near-complete response and very good partial response, see Policy Guidelines).
Tandem transplantation with an initial round of autologous hematopoietic stem-cell transplantation followed by a non-marrow-ablative conditioning regimen and allogeneic hematopoietic stem-cell transplantation (i.e., reduced-intensity conditioning transplant) may be considered medically necessary to treat newly diagnosed multiple myeloma patients.
Allogeneic hematopoietic stem-cell transplantation, myeloablative or nonmyeloablative, as upfront therapy of newly diagnosed multiple myeloma or as salvage therapy, is considered investigational.
Autologous hematopoietic stem-cell transplantation may be considered medically necessary to treat disseminated POEMS syndrome. (see Policy Guidelines)
Allogeneic and tandem hematopoietic stem-cell transplantation are considered investigational to treat POEMS syndrome.
POLICY EXCEPTIONSFor Federal Employee Program (FEP) subscribers, the Service Benefit Plan includes specific conditions in which autologous or allogeneic blood or marrow stem cell transplants would be considered eligible for coverage.
For State and School Employee subscribers, all bone marrow/stem cell transplants must be certified as medically necessary by the Plan’s Utilization Review Vendor. No benefits will be provided for any transplant procedure unless prior approval for the transplant is obtained.
POLICY GUIDELINESThe International Working Group on Myeloma has updated the European Group for Blood and Marrow Transplant (EBMT) criteria to describe a complete response to MM therapy. The criteria include negative immunofixation on the serum and urine; disappearance of soft tissue plasmacytomas; and, 5% or fewer plasma cells in bone marrow aspiration.
Patients with disseminated POEMS syndrome may have diffuse sclerotic lesions or disseminated bone marrow involvement.
Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Nervous/Mental Conditions, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:
A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and
B. appropriate with regard to standards of good medical practice; and
C. not solely for the convenience of the Member, his or her Provider; and
D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.
For the definition of Medically Necessary, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.
Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
POLICY HISTORY8/1998: Approved by Medical Policy Advisory Committee (MPAC)
2/11/2002: Investigational definition added
5/8/2002: Type of Service and Place of Service deleted
3/18/2003: HCPCS G0266, G0267 added covered codes, CPT code 38242 added non-covered codes
1/27/2004: Policy reviewed, policy title "Tandem High Dose Chemoradiotherapy with Autologous Stem Cell Support for Multiple Myeloma" renamed "Single or Tandem Courses of High-Dose Chemotherapy plus Hematopoietic Stem-Cell Support to Treat Multiple Myeloma", Description, Policy, and Policy Guidelines sections aligned with BCBSA policy # 8.01.17, See "High-Dose Chemotherapy with Hematopoietic Stem-Cell Support for Malignancies" medical policy for dates of service prior to 1-27-2004
3/25/2004: Reviewed by MPAC, "Tandem high-dose chemotherapy with autologous stem-cell support is considered investigational to treat multiple myeloma." changed to "Tandem high-dose chemoradiotherapy with autologous stem-cell support may be considered medically necessary to treat newly diagnosed or responsive multiple myeloma.", "An initial course of high-dose chemotherapy with autologous stem-cell support followed by non marrow-ablative chemotherapy and allogeneic stem-cell support (i.e. "mini transplant") is considered investigational to treat multiple myeloma" added
7/19/2004: Code Reference section updated
11/18/2004: Reviewed by MPAC, “An intial course of high-dose chemotherapy with autologous stem-cell support followed by non marrow-ablative chemotherapy and allogeneic stem-cell support (i.e. “mini transplant”) is considered investigational to treat multiple myeloma.” changed to medically necessary
7/11/2005: Code Reference section updated, CPT code 38204, 86812, 86813, 86816, 86817, 86821, 86822 moved from covered codes to non-covered codes, CPT code 38230 added covered codes, HCPCS G0355, G0356, G0357, G0358, G0359, G0360, G0361, G0362, G0363, G0364 added covered codes, HCPCS J9000-J9999 statement added to HCPCS and all separately listed codes deleted
10/21/2005: Code reference section updated, ICD9 code 41.01, 41.09 added to the covered table, 41.02, 41.03 added to the non-covered table, ICD9 procedure code 41.04, 99.79 description revised
03/17/2006: Coding updated. CPT4/HCPCS 2006 revisions added to policy.
12/20/2007: Coding updated per 2008 CPT/HCPCS revisions
9/11/2008: Annual ICD-9 updates applied
04/27/2010: Policy title changed from “Single or Tandem Courses of High Dose Chemotherapy plus Hematopoietic Stem Cell Support to Treat Multiple Myeloma” to “Hematopoietic Stem-Cell Transplantation for Multiple Myeloma.” Changed “Stem-Cell Support” to “Stem-Cell Transplantation” throughout policy. Policy description updated regarding prevalence of disease and treatment approaches. Added the prior authorization requirement to the policy statement. Policy statement updated regarding situations when autologous SCT may be indicated for patients with primary progressive myeloma, and statement regarding allogeneic HSCT being investigational as initial or salvage therapy was reworded. FEP and State and School Employees verbiage added to the Policy Exceptions section. Added new CPT codes 86825 and 86826 to the non-covered table. Deleted CPT codes 96400, 96414, 96520, 96530, and 96545 from the code section as these codes were deleted on 12/31/2005. Deleted HCPCS G0355, G0356, G0357, G0358, G0359, G0360, G0361, and G0362 from the code section as these codes were deleted on 12/31/2005. Deleted HCPCS G0265, G0266, and G0267 from the code section as these codes were deleted on 12/31/2007. Updated policy source section.
06/21/2011: Added "in the tandem sequence" to the medically necessary tandem autologous-autologous policy statement.
05/09/2012: Policy reviewed; no changes.
10/30/2013: Policy title changed from "Hematopoietic Stem Cell Transplantation for Multiple Myeloma" to "Hematopoietic Stem-Cell Transplantation for Plasma Cell Dyscrasias, Including Multiple Myeloma and POEMS Syndrome." Updated policy description regarding POEMS Syndrome and added the following policy statements: Autologous hematopoietic stem-cell transplantation may be considered medically necessary to treat disseminated POEMS syndrome. Allogeneic and tandem hematopoietic stem-cell transplantation are considered investigational to treat POEMS syndrome.
10/23/2014: Policy reviewed; description updated. Policy statement unchanged. Policy guidelines updated to add the EBMT criteria to describe a complete response to MM therapy.
08/27/2015: Code Reference section updated to add ICD-10 codes, updated the code descriptions for 38240 and 38242; removed deleted HCPCS code G0363, and removed deleted code CPT 96445 and replaced with CPT code 96446.
12/11/2015: Policy description updated regarding multiple myeloma. Policy statements unchanged. Policy guidelines updated to add medically necessary and investigative definitions.
05/25/2016: Policy number added.
SOURCE(S)Blue Cross Blue Shield Association policy # 8.01.17
CODE REFERENCEThis may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.