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Prepared by: Gautam Hariish
Multiple Myeloma
Introduction
 Myeloma is a malignant proliferation of neoplastic plasma cells of B-cell
lineage.
 Arises as a result of multiple genetic mutations of plasma cells and the
immunoglobulins they produce.
 These tumours can arise de novo or from the pre malignant condition
monoclonal gammopathy of undetermined significance (MGUS).
 Diagnosis depends on detection of para-proteins in plasma or urine, bone
marrow biopsy, evidence of end organ/bone damage.
Pathophysiology
Epidemiology
 Most common primary malignant lesion arising in bone.
 Typically aged 45-65 .median age at diagnosis is 70 years.
 Males to female ratio approximately 3:2.
 Most commonly arises in marrow containing bones of the
vertebrae, pelvis, femur.
Clinical Features
Physical examination
 Eyes: retinal hemorrhage, exudative macular detachment or
cotton wool spots
 Pallor due to anemia
 Ecchymoses or purpura due to thrombocytopenia
 Bony tenderness
 Nuerology: sensory level change, neuropathy, myopathy, Tinel
sign or Phalen sign due to carpal tunnel compression
secondary to amyloid deposition
 Extramedullary plasmacytoma
 Hepatosplenomegaly
 Cardiomegaly
 Signs of amyloidosis: shoulder pad sign, macroglossia, typical skin
lesions(waxy papules or nodules)
X-rays
 Affected bones show diffuse osteoporosis or lytic lesions
 Biconcave vertebral bodies and collapse of vertebra
 Punched out lesion in skull and pelvis
Treatment
 Immediate pain control with analgesics.
 General supportive measures- correcting the fluid imbalance such as
hypercalcemia.
 Pathological limb fractures should be managed with internal fixation.
 Surgery has supportive effect on management, long time survival and
quality of life.
 Aim is to decompress or stabilize vertebral fractures with instability or
neurologic compression, to stabilize pathological fractures and to reduce
pain.
Treatment
 Treatment comprises alkylating chemotherapy, a steroid such as
dexamethasone or prednisolone and and immunomodulatory agents
such as thalidomide.
 Autologous stem cell transplantation involves bone marrow harvest, high
dose chemotherapy to produce myelosuppresion and transfusion with
stem cells(standard of care in patients up to 65 years).
 Bisphosphonates given to prevent fractures and control hypercalcaemia..
Prognosis
 Adverse prognostic factors include anemia, hypercalcemia, renal failure,
hyperuricemia, hypoalbuminemia and elevated beta-2 macroglobulin
level.
 Prognosis is highly variable.
 Overall estimated survival improved to 35% 5-year survival and 17% 10-
year survival with advent of new treatments.

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Multiple myeloma

  • 1. Prepared by: Gautam Hariish Multiple Myeloma
  • 2. Introduction  Myeloma is a malignant proliferation of neoplastic plasma cells of B-cell lineage.  Arises as a result of multiple genetic mutations of plasma cells and the immunoglobulins they produce.  These tumours can arise de novo or from the pre malignant condition monoclonal gammopathy of undetermined significance (MGUS).  Diagnosis depends on detection of para-proteins in plasma or urine, bone marrow biopsy, evidence of end organ/bone damage.
  • 4. Epidemiology  Most common primary malignant lesion arising in bone.  Typically aged 45-65 .median age at diagnosis is 70 years.  Males to female ratio approximately 3:2.  Most commonly arises in marrow containing bones of the vertebrae, pelvis, femur.
  • 6. Physical examination  Eyes: retinal hemorrhage, exudative macular detachment or cotton wool spots  Pallor due to anemia  Ecchymoses or purpura due to thrombocytopenia  Bony tenderness  Nuerology: sensory level change, neuropathy, myopathy, Tinel sign or Phalen sign due to carpal tunnel compression secondary to amyloid deposition  Extramedullary plasmacytoma
  • 7.  Hepatosplenomegaly  Cardiomegaly  Signs of amyloidosis: shoulder pad sign, macroglossia, typical skin lesions(waxy papules or nodules)
  • 8.
  • 9.
  • 10.
  • 11.
  • 12. X-rays  Affected bones show diffuse osteoporosis or lytic lesions  Biconcave vertebral bodies and collapse of vertebra  Punched out lesion in skull and pelvis
  • 13.
  • 14.
  • 15.
  • 16. Treatment  Immediate pain control with analgesics.  General supportive measures- correcting the fluid imbalance such as hypercalcemia.  Pathological limb fractures should be managed with internal fixation.  Surgery has supportive effect on management, long time survival and quality of life.  Aim is to decompress or stabilize vertebral fractures with instability or neurologic compression, to stabilize pathological fractures and to reduce pain.
  • 17. Treatment  Treatment comprises alkylating chemotherapy, a steroid such as dexamethasone or prednisolone and and immunomodulatory agents such as thalidomide.  Autologous stem cell transplantation involves bone marrow harvest, high dose chemotherapy to produce myelosuppresion and transfusion with stem cells(standard of care in patients up to 65 years).  Bisphosphonates given to prevent fractures and control hypercalcaemia..
  • 18. Prognosis  Adverse prognostic factors include anemia, hypercalcemia, renal failure, hyperuricemia, hypoalbuminemia and elevated beta-2 macroglobulin level.  Prognosis is highly variable.  Overall estimated survival improved to 35% 5-year survival and 17% 10- year survival with advent of new treatments.