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Multiple Myeloma

Rick Allen. Multiple Myeloma. A malignant proliferation of plasma cells derived from a single clone, with multifocal involvement of the skeleton. What is it?. 1,115 Aussies diagnosed every year Risk increases w. age : 80% are > 60 y.o . Men > women Black 2x > white

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Multiple Myeloma

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  1. Rick Allen Multiple Myeloma

  2. A malignant proliferation of plasma cells derived from a single clone, with multifocal involvement of the skeleton. What is it?

  3. 1,115 Aussies diagnosed every year Risk increases w. age : 80% are > 60 y.o. Men > women Black 2x > white Familial link (4x increased risk) 1% of all US cancers, 10% of haemotological cancers Epidemiology

  4. Cause is unknown, however there is evidence for genetic issues; • 11q14 and 17p13 deletions (serious) • 11q abnormalities • T(11;14)(q13;q32) , cyclin D1 (cell cycle regulatory gene) (less serious) • T(4;14)(p16;q32) heavy chain gene and tyrosine kinase receptor controls cell proliferation • Mys, ras, p53 and Rb-1 mutations Aetiology

  5. Plasma cell attaches to bone marrow stromal cell (handy because…) Monoclonal Ig (M component) is produced; potentially with excess heavy/light chains (urine) Bone destruction (↑ RANKL on OB  ↑ OC act., inhibition of OB) IL-6, IGF-1 the main players Pathophysiology

  6. Pathophysiology

  7. Are due to: • Plasma cell growth in tissue • Excessive defective Ig production • Normal humoral immunity suppression Clinical Features

  8. Bone resorption •  pathologic fractures and bone pain (usually precipitated by movement). Generalized osteoperosis • hypercalcaemia  neurologic symptoms and renal dysfunction • ↓ normal Ig production •  Recurrent bacterial infection • Cell immunity not affected • Ig breakdown increased • IgA ↑ blood viscosity  headaches, retinopathy, fatigue • Renal failure • Multifactorial cause, but primarily due to Bence-Jones proteinuria • Toxic  atrophy of tubal epithelia, pyelonephritis • Anaemia • Due to marrow involvement. Normocytic, normochromic. Pancytopenia. Clinical Features

  9. Clinical Features

  10. Destructive plasma cell tumours in axial skeleton Medullary cavity  erodes spongy bone  destroys cortical bone. Lesions 1-4cm diameter Soft, gelatinous, red tumour mass Elsewhere, ↑ marrow plasma cellularity Morphology

  11. Morphology

  12. Radiograph and lab results • 24hr urine to find Bence-Jones bodies • Electrophoresis to determine monoclonal Ig/light chains • X-rays of osteolytic lesions: require bone marrow examination to confirm. Diagnosing

  13. Some lymphomas and leukaemias (CLL) can also produce M components. Differentials

  14. Systemic treatment + symptomatic treatment Cytotoxic agents (proteasome inhibitors) Combination chemo: alters myeloma and stromal cell interaction. Inhibits angiogenesis Bisphosphonates  bone and Ca Transplant: prolongs but no cure Radiotherapy for bone pain Treatment/Management

  15. Median survival 4-6 years Multiple bony lesions  6-12 months Death usually due to either renal failure or infection Prognosis

  16. Plasmacytoma • Localised myeloma • Have the potential to spread. Easier to treat if found in soft tissue. • Monoclonal Gammopathy of Uncertain Significance (MGUS) • Same genetic abnormalities as MM • Asymptommatic w. elevated M components. • Progression to MM ~1%. Unpredictable. Should know a bit about…

  17. Robbins and Cotran, pp609-611 Harrisons, pp701-706 Underwood, pp667-669 Leukaemia association of Australia Up to Date References

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