Home Oncology Multiple Myeloma & Plasma Cell Disorders

Multiple Myeloma & Plasma Cell Disorders

📋 Key Information Summary

📋
  • Multiple myeloma is a clonal plasma cell malignancy characterised by CRAB criteria: Calcaemia (hypercalcaemia), Renal insufficiency, Anaemia, and Bone lesions — these represent end-organ damage and mandate treatment initiation.
  • The International Staging System (ISS) and Revised ISS (R-ISS) incorporating β₂-microglobulin, albumin, LDH, and cytogenetic risk stratify patients into standard-, intermediate-, and high-risk groups.
  • Key investigations include serum protein electrophoresis (SPEP) with immunofixation, serum free light chain (sFLC) assay, 24-hour urine Bence Jones protein, bone marrow biopsy with FISH/cytogenetics, and whole-body low-dose CT or PET-CT.
  • Induction therapy in transplant-eligible patients typically comprises a bortezomib-based triplet (e.g., bortezomib–lenalidomide–dexamethasone [VRd]) followed by autologous stem cell transplantation (ASCT).
  • In transplant-ineligible patients, VRd-lite (modified dosing) or daratumumab-containing regimens (D-VMP, DRd) are first-line per Australian guidelines.
  • Proteasome inhibitors (bortezomib, carfilzomib) and immunomodulatory drugs (lenalidomide, pomalidomide) form the backbone of myeloma therapy; monoclonal antibodies (daratumumab, isatuximab) are increasingly incorporated.
  • Bortezomib subcutaneous administration reduces peripheral neuropathy; schedule weekly (days 1, 8, 15) rather than twice-weekly to improve tolerability.
  • Lenalidomide is PBS-listed as Authority Required for maintenance post-ASCT and relapsed/refractory myeloma; dose adjustment is essential for renal impairment (CrCl <60 mL/min).
  • Bisphosphonate therapy (zoledronic acid or pamidronate) is indicated for all patients with myeloma bone disease; denosumab is an alternative if renal impairment precludes bisphosphonates.
  • Tumour lysis syndrome prophylaxis and thromboprophylaxis with lenalidomide-containing regimens are critical supportive measures.
  • Aboriginal and Torres Strait Islander peoples may present with later-stage disease and have higher rates of renal complications; culturally safe, community-based follow-up improves outcomes.
  • Smouldering (asymptomatic) myeloma requires monitoring only unless high-risk features are present; the 20/2/20 criteria help identify candidates for early intervention.

Introduction & Australian Epidemiology

Multiple myeloma is a malignant neoplasm of terminally differentiated B-lymphocytes (plasma cells) that accumulate in the bone marrow, producing monoclonal immunoglobulin (M-protein) and/or free light chains. It accounts for approximately 10% of all haematological malignancies and remains incurable in the majority of patients, although recent therapeutic advances have substantially improved survival.

In Australia, myeloma is the second most common haematological malignancy. The Australian Institute of Health and Welfare (AIHW) reports approximately 1,800–2,000 new diagnoses annually, with an age-standardised incidence rate of around 7.5 per 100,000. Median age at diagnosis is 69 years, with a slight male predominance (1.4:1). Five-year relative survival has improved from approximately 30% in the early 2000s to over 55% in recent cohorts, attributable to proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies, and improved supportive care.

Aboriginal and Torres Strait Islander Australians have a higher incidence of myeloma and are more likely to present with advanced disease, renal impairment, and hypercalcaemia. Barriers to timely diagnosis and specialist referral contribute to these disparities.

The diagnostic and therapeutic landscape continues to evolve with the introduction of chimeric antigen receptor (CAR) T-cell therapies (ide-cel, cilta-cel) and bispecific antibodies (teclistamab, elranatamab) for relapsed/refractory disease, though access in Australia remains largely through clinical trials and special access schemes.

Pathogenesis & CRAB Criteria

Pathogenesis

Multiple myeloma arises from clonal expansion of post-germinal centre plasma cells within the bone marrow. The pathogenesis involves a multi-step process:

  • Monoclonal gammopathy of undetermined significance (MGUS): Present in ~3.5% of adults >50 years; progresses to myeloma at ~1% per year.
  • Smouldering multiple myeloma (SMM): Intermediate asymptomatic stage; 10% risk of progression in the first 5 years, declining thereafter.
  • Symptomatic multiple myeloma: Characterised by end-organ damage defined by CRAB criteria or specific biomarkers of malignancy.

Key genetic events include primary translocations involving the immunoglobulin heavy chain locus (t(11;14), t(4;14), t(14;16)), hyperdiploidy, and secondary mutations in NRAS, KRAS, TP53, and DIS3. The bone marrow microenvironment — including osteoclasts, osteoblasts, and stromal cells — plays a critical role in disease progression, bone destruction, and drug resistance.

CRAB Criteria — Defining End-Organ Damage

C
Hypercalcaemia
Corrected serum calcium >2.75 mmol/L or >0.25 mmol/L above the upper limit of normal. Caused by osteoclast activation via RANK-L upregulation.
IV saline ± bisphosphonate; treat underlying myeloma
R
Renal Insufficiency
Serum creatinine >177 µmol/L or CrCl <40 mL/min. Light-chain cast nephropathy is the most common mechanism; hypercalcaemia and dehydration are contributory.
Hydration, avoid nephrotoxins, rapid anti-myeloma therapy; consider plasma exchange for severe cast nephropathy
A
Anaemia
Haemoglobin <100 g/L or >20 g/L below normal. Normochromic, normocytic; erythropoietin suppressed; bone marrow infiltration by plasma cells.
Erythropoiesis-stimulating agents if Hb <100 g/L; transfuse for symptomatic anaemia; treat underlying myeloma
B
Bone Lesions
One or more lytic bone lesions on skeletal survey, whole-body low-dose CT, or PET-CT. Osteolytic lesions rarely heal; pathological fractures common at vertebrae, ribs, pelvis, long bones.
Bisphosphonates or denosumab; orthopaedic referral for impending fracture; radiotherapy for localised pain
⚠️
Updated diagnostic criteria (IMWG 2014): In addition to CRAB criteria, any one of the following biomarkers can define myeloma requiring therapy: clonal bone marrow plasma cells ≥60%, serum involved/uninvolved free light chain ratio ≥100 (involved level ≥100 mg/L), or >1 focal lesion on MRI (each ≥5 mm). These "SLiM" criteria allow earlier treatment before irreversible organ damage occurs.

Investigations (SPEP, Biopsy & Imaging)

Laboratory Investigations

Essential
Serum Protein Electrophoresis (SPEP) with Immunofixation
Detects and characterises M-protein (IgG, IgA, IgD, IgE, light-chain only). Immunofixation identifies heavy and light chain type. Quantify M-protein for response assessment. Available in all Australian pathology services.
Essential
Serum Free Light Chain (sFLC) Assay
Measures κ and λ free light chains; calculates the κ/λ ratio. Essential for diagnosis and monitoring of light-chain myeloma and non-secretory myeloma. MBS item available through all major laboratories.
Essential
24-Hour Urine Protein Electrophoresis with Immunofixation
Quantifies Bence Jones proteinuria; required for response assessment per IMWG criteria. Urine dipstick alone is insufficient — must request electrophoresis.
Essential
Bone Marrow Aspirate & Trephine Biopsy
Confirms clonal plasma cell infiltration (percentage, morphology), with flow cytometry (CD38+, CD138+, CD56+/-, CD19−), FISH for t(4;14), t(14;16), del(17p), gain 1q, and conventional cytogenetics. Sufficient trephine length ≥2 cm for accurate assessment.
Essential
Full Blood Examination, Renal Function, Corrected Calcium, LDH, β₂-microglobulin, Albumin
Required for CRAB assessment, ISS/R-ISS staging, and baseline organ function. β₂-microglobulin and albumin are core ISS staging parameters.

Imaging

Essential
Whole-Body Low-Dose CT (WB-LDCT)
Now the preferred initial imaging modality (replacing skeletal survey) per IMWG 2019 guidelines. Detects osteolytic lesions ≥5 mm, assesses fracture risk. MBS-rebated for myeloma staging. Available at most tertiary centres.
Available
PET-CT (¹⁸F-FDG)
Superior sensitivity for extramedullary disease and focal lesions; assesses metabolic response to therapy. Indicated for initial staging and detection of extramedullary relapse. Limited availability in regional Australia; may require referral to metropolitan centres.
Available
MRI (Whole-Body or Spine)
Gold standard for detecting bone marrow infiltration and cord compression. Superior to CT for detecting diffuse marrow involvement. Indicated when PET-CT is equivocal or for spinal cord assessment. Available at all major centres.

Additional Investigations at Diagnosis

  • LDH: Elevated LDH indicates aggressive disease biology and extramedullary involvement; required for R-ISS staging.
  • Serum viscosity: If symptomatic hyperviscosity (IgA and IgG3 subtypes most likely); treat with plasmapheresis if symptomatic.
  • Baseline ECG and cardiac assessment: Prior to carfilzomib or anthracycline-containing regimens.
  • Hepatitis B serology: Mandatory before immunosuppressive therapy; antiviral prophylaxis if HBsAg positive or HBcAb positive.
  • Immunoglobulin levels: Quantitative IgG, IgA, IgM; monitor for secondary hypogammaglobulinaemia and infection risk.

Staging (ISS) & Prognosis

International Staging System (ISS)

Stage Criteria Median Survival
I β₂-microglobulin <3.5 mg/L and albumin ≥35 g/L 62 months
II Neither stage I nor III 44 months
III β₂-microglobulin ≥5.5 mg/L 29 months

Revised International Staging System (R-ISS)

The R-ISS incorporates cytogenetic risk and LDH into the ISS, providing more refined prognostication:

R-ISS Stage Criteria 5-Year OS
I ISS I + standard-risk cytogenetics + normal LDH ~82%
II Neither I nor III ~62%
III ISS III + high-risk cytogenetics [t(4;14), t(14;16), del(17p)] or elevated LDH ~40%

Cytogenetic Risk Stratification

Standard Risk
Favourable Cytogenetics
Hyperdiploidy, t(11;14). Median survival with modern therapy >7 years.
Intermediate Risk
t(4;14) Alone
Without del(17p) or TP53 mutation; median survival ~4–5 years with contemporary regimens.
High Risk
Del(17p), t(14;16), TP53 Mutation, gain 1q (≥3 copies)
Median survival <3 years historically; novel agents and tandem transplant improving outcomes. Consider clinical trial enrolment.
ℹ️
R-ISS III implications: Patients with R-ISS III disease, particularly those with del(17p)/TP53 mutations, should be considered for triplet/quadruplet induction, consolidation, prolonged maintenance, and clinical trial enrolment. Autologous transplant remains standard of care in eligible patients regardless of risk status.

Management (Bortezomib, IMiDs & ASCT)

Treatment Paradigm Overview

Myeloma management is stratified by transplant eligibility (typically age <70–75, adequate organ function, ECOG 0–2) and risk status. All patients require assessment at a multidisciplinary myeloma clinic with access to autologous stem cell transplantation.

Induction Therapy — Transplant-Eligible Patients

The current Australian standard of care induction is bortezomib, lenalidomide, and dexamethasone (VRd) for 4–6 cycles:

💊
Bortezomib
Velcade® · Proteasome inhibitor
Adult dose 1.3 mg/m² SC on days 1, 8, 15 (weekly schedule preferred) or days 1, 4, 8, 11 (twice weekly, 21-day cycle)
Route Subcutaneous (preferred — reduces peripheral neuropathy vs IV)
Key side effects Peripheral neuropathy (dose-limiting), thrombocytopenia, herpes zoster reactivation (valaciclovir prophylaxis essential), GI symptoms
Renal adjustment No dose adjustment required; safe in dialysis patients
PBS status ⚠ PBS Authority Required
💊
Lenalidomide
Revlimid® · Immunomodulatory drug (IMiD)
Adult dose 25 mg PO daily on days 1–21 of each 28-day cycle (induction); 10–15 mg daily as maintenance
Route Oral
Key side effects Venous thromboembolism (mandatory thromboprophylaxis — aspirin or LMWH), myelosuppression (neutropenia most common), fatigue, rash, teratogenicity
Renal adjustment CrCl 30–59 mL/min: 10 mg daily; CrCl <30 mL/min (non-dialysis): 15 mg every 48 hours; CrCl <30 mL/min (dialysis): 5 mg daily on dialysis days
PBS status ⚠ PBS Authority Required
💊
Dexamethasone
Multiple brands · Corticosteroid
Adult dose 40 mg PO weekly (days 1, 8, 15, 22; reduce to 20 mg for patients >75 years); can use 20 mg on bortezomib days + 10 mg on non-bortezomib days
Route Oral
Key side effects Insomnia, hyperglycaemia, infections (especially with concurrent immunosuppression), proximal myopathy, adrenal suppression
PBS status ✔ PBS General Benefit
⚠️
Thromboprophylaxis with lenalidomide: All patients receiving lenalidomide require venous thromboembolism (VTE) prophylaxis. Low-risk patients: aspirin 100 mg PO daily. High-risk patients (prior VTE, immobility, obesity, concurrent erythropoietin, or doxorubicin): enoxaparin 40 mg SC daily or warfarin (INR 2–3) or a DOAC per institutional protocol.

Autologous Stem Cell Transplantation (ASCT)

ASCT remains the standard of care for transplant-eligible patients in Australia. It is performed following 4–6 cycles of induction therapy and PBSC mobilisation with cyclophosphamide + G-CSF or G-CSF ± plerixafor.

1
Induction (4–6 cycles VRd)
Achieve best partial response or better prior to transplant. Reassess with SPEP, sFLC, and bone marrow biopsy.
2
Stem Cell Mobilisation & Collection
Cyclophosphamide 2–3 g/m² + G-CSF 10 µg/kg/day; or G-CSF alone ± plerixafor. Target CD34+ cell dose ≥2 × 10⁶/kg (ideally ≥4 × 10⁶/kg for tandem transplant consideration).
3
Conditioning & Transplant
Melphalan 200 mg/m² IV (reduce to 140 mg/m² if age >65, CrCl <60 mL/min, or significant comorbidities). Infusion of cryopreserved autologous stem cells (Day 0).
4
Consolidation & Maintenance
Post-ASCT consolidation: 2 cycles of VRd (for high-risk disease). Maintenance: lenalidomide 10–15 mg daily until progression or intolerance — PBS Authority Required.

Induction Therapy — Transplant-Ineligible Patients

For patients not eligible for ASCT (typically >75 years, significant comorbidities), the following regimens are recommended:

  • Daratumumab–bortezomib–melphalan–prednisolone (D-VMP): Daratumumab 16 mg/kg IV weekly × 6 weeks (cycle 1), then every 3 weeks × 8 cycles, then every 4 weeks maintenance. Bortezomib 1.3 mg/m² SC weekly × 9 cycles. Melphalan 9 mg/m² PO days 1–4. Prednisolone 60 mg/m² PO days 1–4. PBS Authority Required for daratumumab.
  • Daratumumab–lenalidomide–dexamethasone (DRd): Daratumumab + lenalidomide 25 mg days 1–21 + dexamethasone 40 mg weekly. Increasingly preferred due to superior progression-free survival (MAIA trial).
  • Bortezomib–lenalidomide–dexamethasone (VRd-lite): Modified dosing for elderly: bortezomib 1.3 mg/m² SC weekly, lenalidomide 15 mg days 1–21, dexamethasone 20 mg weekly.

Relapsed/Refractory Myeloma

Treatment at relapse depends on the number of prior lines, agents used, duration of prior response, and transplant eligibility. Key regimens include:

💊
Pomalidomide
Pomalyst® · Second-generation IMiD
Adult dose 4 mg PO daily on days 1–21 of 28-day cycle + dexamethasone 40 mg weekly (20 mg if >75 years)
Key side effects Myelosuppression, VTE (thromboprophylaxis required), fatigue, neuropathy (less than lenalidomide)
Renal adjustment No formal dose adjustment; use with caution in severe renal impairment
PBS status ⚠ PBS Authority Required
💊
Carfilzomib
Kyprolis® · Second-generation proteasome inhibitor
Adult dose 20 mg/m² IV day 1 cycle 1 (escalate to 56 mg/m² IV on days 1, 8, 15 of 28-day cycles) + dexamethasone ± daratumumab or isatuximab (IKEMA/ASPIRE trials)
Key side effects Cardiac toxicity (monitor BP and LVEF), dyspnoea, hypertension, thrombocytopenia
PBS status ⚠ PBS Authority Required
💊
Isatuximab
Sarclisa® · Anti-CD38 monoclonal antibody
Adult dose 10 mg/kg IV weekly × 4 weeks, then every 2 weeks + carfilzomib–dexamethasone (IKEMA) or pomalidomide–dexamethasone (ICARIA)
Key side effects Infusion reactions (premedicate with paracetamol, antihistamine, corticosteroid), neutropenia, infections, second primary malignancies
PBS status ⛔ Not PBS Listed

Supportive Care — Mandatory for All Patients

  • Bisphosphonates: Zoledronic acid 4 mg IV every 4 weeks (or monthly for up to 2 years, then 3-monthly per MRC Myeloma IX data). Adjust for renal function (CrCl <30 mL/min: avoid or halve dose). Pamidronate 90 mg IV is an alternative. PBS General Benefit.
  • Denosumab: 120 mg SC every 4 weeks. Alternative to bisphosphonates when renal impairment precludes zoledronic acid. Monitor for hypocalcaemia. PBS Authority Required for myeloma bone disease.
  • Infection prophylaxis: Valaciclovir 500 mg PO daily for herpes zoster prophylaxis during bortezomib therapy; consider co-trimoxazole 480 mg PO daily for PCP prophylaxis with significant immunosuppression; IVIg if recurrent bacterial infections and IgG <4 g/L.
  • Erythropoiesis-stimulating agents (ESAs): If Hb <100 g/L and on active therapy; combine with iron supplementation if iron-deficient. PBS Authority Required.
  • Skeletal protection: Weight-bearing exercise, calcium and vitamin D supplementation, physiotherapy, vertebroplasty for vertebral compression fractures.
  • Vaccination: Annual influenza, pneumococcal (Prevenar 13 then Pneumovax 23), COVID-19 boosters; avoid live vaccines. COVID-19 vaccination timing relative to daratumumab should be coordinated with treating team.
  • Pain management: Multimodal analgesia; radiotherapy for localised bone pain; surgical stabilisation for impending or actual pathological fractures.

Monitoring & Response Assessment

Timepoint Assessment Details
Each cycle SPEP, sFLC, FBC, renal function, calcium Monitor response and toxicity; adjust doses per protocol
Post-induction (cycle 4–6) SPEP, sFLC, urine IFE, bone marrow biopsy (if CR suspected) IMWG response criteria: sCR, CR, VGPR, PR, SD, PD
Post-ASCT (Day +100) SPEP, sFLC, bone marrow biopsy, PET-CT (optional) Assess depth of response; decide on consolidation/maintenance
During maintenance SPEP, sFLC, FBC, renal function every 1–3 months MRD assessment (flow cytometry or NGS) increasingly used in trials
Suspected relapse SPEP, sFLC, PET-CT or MRI, bone marrow biopsy Biochemical relapse (25% M-protein increase) vs clinical relapse (CRAB); guide retreatment decisions
🚨
Emergency presentations: Myeloma patients may present with spinal cord compression (urgent MRI + dexamethasone 16 mg IV + radiotherapy within 24 hours), hypercalcaemic crisis (IV saline 4–6 L/24h + zoledronic acid ± calcitonin), acute renal failure (hydration, avoid nephrotoxins, urgent anti-myeloma therapy), or hyperviscosity syndrome (plasmapheresis). These require emergent haematology/oncology referral.

Special Populations

🤰 Pregnancy
Myeloma in pregnancy is extremely rare (median age 69 years).
Lenalidomide and pomalidomide are absolutely contraindicated (teratogenic Category X). Bortezomib has limited pregnancy data but may be used if essential. Dexamethasone can be used. Multidisciplinary team with obstetric input essential.
👶 Paediatric
Multiple myeloma is exceptionally rare in patients <30 years.
Paediatric plasma cell neoplasms should prompt consideration of immunodeficiency or POEMS syndrome. Refer to paediatric haematology-oncology centre. Dosing should follow paediatric oncology protocols.
👴 Elderly (≥75 years)
Reduced-dose dexamethasone (20 mg weekly).
Higher infection risk with aggressive regimens. VRd-lite or D-VMP preferred. Lenalidomide dose adjustment for renal function. Assess frailty using IMWG frailty score to guide treatment intensity. Consider geriatric assessment for functional status.
🫘 Renal Impairment
Bortezomib — no dose adjustment; safe in dialysis.
Lenalidomide — dose reduce per CrCl (see above). Avoid zoledronic acid if CrCl <30 mL/min; use denosumab or pamidronate. High fluid intake (≥2.5 L/day) to reduce light-chain nephrotoxicity. Rapid anti-myeloma therapy is critical for reversible renal failure.
🫁 Hepatic Impairment
Dexamethasone dose reduction for Child-Pugh B/C.
Bortezomib: start at 0.7 mg/m² for moderate-severe hepatic impairment (Child-Pugh B/C). Lenalidomide: no specific hepatic dose adjustment but use cautiously. Monitor for hepatotoxicity with all agents.
🛡️ Immunocompromised
Hypogammaglobulinaemia is intrinsic to myeloma.
Infection is the leading cause of early death. IVIg replacement for IgG <4 g/L with recurrent infections. Aggressive antimicrobial prophylaxis. Vaccination responses impaired — avoid live vaccines. COVID-19 vaccination timing with daratumumab requires coordination.

Aboriginal and Torres Strait Islander Health Considerations

Aboriginal and Torres Strait Islander Health

Multiple myeloma has a higher age-standardised incidence in Aboriginal and Torres Strait Islander Australians compared to non-Indigenous Australians. ATSI patients are more likely to present with advanced disease, higher rates of renal impairment, hypercalcaemia, and anaemia at diagnosis, contributing to poorer outcomes.

Diagnostic delay
Later presentation with more advanced CRAB features; may reflect barriers to accessing pathology, imaging, and specialist referral in rural and remote communities. Point-of-care testing and telehealth haematology consultations can reduce time to diagnosis.
Renal burden
Higher background rates of chronic kidney disease in ATSI populations compound myeloma-related renal impairment. Aggressive hydration, early nephrology involvement, and close monitoring of renal function during therapy are essential.
Geographic access
Autologous stem cell transplantation is available only at major metropolitan centres (Sydney, Melbourne, Brisbane, Adelaide, Perth). Long-distance travel, separation from family, and cultural disconnection are significant barriers. Patient-assisted travel schemes (PATS) and Aboriginal liaison officers can improve engagement.
Treatment adherence
Oral therapies (lenalidomide, pomalidomide) require sustained adherence. Support from community health workers, remote pharmacy services, and culturally appropriate education materials improves medication compliance. Dose administration aids (DAAs) should be considered.
Infection risk
Higher background rates of hepatitis B and C, rheumatic heart disease, and chronic lung disease in ATSI communities increase the risk of infections during immunosuppressive myeloma therapy. Mandatory hepatitis B screening and comprehensive infection prophylaxis are critical.
Cultural safety
End-of-life discussions, bone marrow biopsy, and prolonged hospitalisation require culturally sensitive communication. Involve Aboriginal health workers and community elders in care planning. Respect Sorry Business obligations and their impact on treatment schedules.

📚 References

  1. 1. Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15(12):e538–e548. doi:10.1016/S1470-2045(14)70442-5
  2. 2. Palumbo A, Avet-Loiseau H, Oliva S, et al. Revised International Staging System for Multiple Myeloma: a report from International Myeloma Working Group. J Clin Oncol. 2015;33(26):2863–2869. doi:10.1200/JCO.2015.61.2267
  3. 3. Durie BGM, Hoering A, Abidi MH, et al. Bortezomib with lenalidomide and dexamethasone versus lenalidomide and dexamethasone alone in patients with newly diagnosed myeloma without intent for immediate autologous stem-cell transplant (SWOG S0777): a randomised, open-label, phase 3 trial. Lancet. 2017;389(10068):519–527. doi:10.1016/S0140-6736(16)31594-X
  4. 4. Facon T, Kumar S, Plesner T, et al. Daratumumab plus lenalidomide and dexamethasone for untreated myeloma. N Engl J Med. 2019;380(22):2104–2115. doi:10.1056/NEJMoa1817249
  5. 5. Mateos MV, Dimopoulos MA, Cavo M, et al. Daratumumab plus bortezomib, melphalan, and prednisone for untreated myeloma. N Engl J Med. 2018;378(6):518–528. doi:10.1056/NEJMoa1714678
  6. 6. Attal M, Lauwers-Cances V, Hulin C, et al. Lenalidomide, bortezomib, and dexamethasone with transplantation for myeloma. N Engl J Med. 2017;376(14):1311–1320. doi:10.1056/NEJMoa1611750
  7. 7. Australian Institute of Health and Welfare (AIHW). Cancer data in Australia: Myeloma. Canberra: AIHW; 2023. Available from: https://www.aihw.gov.au/reports/cancer/cancer-data-in-australia
  8. 8. Cavo M, Gay F, Beksac M, et al. Autologous haematopoietic stem-cell transplantation versus bortezomib–melphalan–prednisone, with or without bortezomib–lenalidomide–dexamethasone consolidation therapy, and lenalidomide maintenance for newly diagnosed multiple myeloma (EMN02/HO95): a multicentre, randomised, open-label, phase 3 study. Lancet Haematol. 2020;7(6):e456–e468. doi:10.1016/S2352-3026(20)30099-5
  9. 9. Dimopoulos MA, Moreau P, Palumbo A, et al. Carfilzomib and dexamethasone versus bortezomib and dexamethasone for patients with relapsed or refractory multiple myeloma (ENDEAVOR): a randomised, phase 3, open-label, multicentre study. Lancet Oncol. 2016;17(1):27–38. doi:10.1016/S1470-2045(15)00464-7
  10. 10. Richardson PG, Oriol A, Beksac M, et al. Pomalidomide, bortezomib, and dexamethasone for patients with relapsed or refractory multiple myeloma previously treated with lenalidomide (OPTIMISMM): a randomised, open-label, phase 3 trial. Lancet Oncol. 2019;20(6):781–794. doi:10.1016/S1470-2045(19)30152-4
  11. 11. Morgan GJ, Davies FE, Gregory WM, et al. First-line treatment with zoledronic acid as compared with clodronic acid in multiple myeloma (MRC Myeloma IX): a randomised controlled trial. Lancet. 2010;376(9757):1989–1999. doi:10.1016/S0140-6736(10)62051-X
  12. 12. National Health and Medical Research Council (NHMRC). National Statement on Ethical Conduct in Human Research. Canberra: NHMRC; 2023 (updated). Relevant to clinical trial participation in myeloma research.
  13. 13. Australian Commission on Safety and Quality in Health Care (ACSQHC). National Safety and Quality Health Service Standards. 2nd ed. Sydney: ACSQHC; 2021. Standards relevant to blood transfusion, medication safety, and infection prevention in myeloma care.
  14. 14. Conyers R, Young S, White C, et al. Cancer in Aboriginal and Torres Strait Islander peoples of Australia: an overview. Aust NZ J Public Health. 2019;43(5):482–486. doi:10.1111/1753-6405.12916
for PBS-listed medicines at participating pharmacies.
Cultural safety
Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.
Medication adherence
Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.
Specific conditions
Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.
Referral pathways
Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

  1. 1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
  2. 2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
  3. 3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
  4. 4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
  5. 5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
  6. 6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
  7. 7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
  8. 8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
  9. 9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
  10. 10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
  11. 11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
  12. 12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
  13. 13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).
for PBS-listed medicines at participating pharmacies.
Cultural safety
Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.
Medication adherence
Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.
Specific conditions
Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.
Referral pathways
Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

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