FN: Multiple Myeloma

Question 1

Epi

Answer 1

M=F
Black>White = 2:1
70yrs

Question 2

Pathogenesis

Answer 2

1. clonal proliferation of plasma cells – monoclonalraised Ig - usually IgG or IgA
2. Clones may also produce free light chain (kappa or lambda): 2/3
3. Clones produce IL-6 whcih inhibits osteoblasts (normal ALP) and activates oteoblasts

Question 3

Free light chains excreted by and seen

Answer 3

Excreted by kidney - urinary BJP

Light chains only seen in plasma in renal failure

Question 4

Symptoms

Answer 4

Osteolytic bone lesions
BM infiltration
Recurrent bacterial infections
Renal impairment

Question 5

Osteolytic bone lesions

Answer 5

1. Backache and bone pain
2. PAthological fractures
3. Vertebral collapse

Question 6

BM infiltration

Answer 6

anaemia
Neutropenia
Thrombocytopenia

Question 7

Recurrent bacterial infections

Answer 7

Neutropenia
Immunoparesis (reduced Ig)
Chemotherapy

Question 8

Renal Impairment

Answer 8

Light chains
Raised Calcium
AL-amyloid

Question 9

complications

Answer 9

Hypercalcaemia
Neurlogical: raised Calcium, compression, amyloid
AKI
Hyperviscosity
AL-amyloid (15%)

Question 10

Investigations

Answer 10

Bloods
Urine
BM biopsy
Xray

Question 11

Bloods show

Answer 11

1. FBC: nromocytic normochromic anaemia
2. Film: rouleaux ± plasma cells ± cytopenias
3. Raised ESR/PV, raised U+Cr, raised Calcium, normal ALP
4. Se electrophoresis: BJP

Question 12

Urine shows

Answer 12

Stix: raised specific gravity (BJP donesnt show)
Electrophoresis: BJP

Question 13

X-ray Skeletal Pathoogy

Answer 13

Punched-out lytic lesions
Pepper-pot skull
Vertebral collapse
Fractures

Question 14

Diagnosis

Answer 14

Clonal BM plasma cells >10%
Presence of se and/or urinary monoclonal protein
End-organ Damage: CRAB

Question 15

CRAB

Answer 15

Calcium
Renal insufficiency
Anaemia (

Question 16

Management

Answer 16

Supportive
Complications Rx
Specific

Question 17

Supportive

Answer 17

Bone pain: Analgesia (avoid NSAIDS) + bisphosphonates
Anaemia: Transfusions and EPO
Renal impairement: ensure good hydration ! dialysis
Infections: broad spectrum Abx ± IVIg if recurrent

Question 18

Complications

Answer 18

Raised Calcium: aggressive hydration, frusemide, bisphosphonates
Cord compression: MRI dexamethasone + local radio
Hyperviscosity: Plasmapheresis (remove light chains)
AKI: rehydration ± dialysis

Question 19

Specific Rx for fit patients

Answer 19

Induction chemo: lenalidomide + low-dose dex

Them allogenic BMT

Question 20

Specific RX for unfit pts.

Answer 20

Chemo only: melphalan + pred + lenalidomide

Bortezomib for relapse

Question 21

Prognosis

Answer 21

Mean survival: 3-5 yrs
Poor prognostic indicators
1. Beta2 microglobulin
Reduced albumin