Haematopoietic

Question 1

Define mutliple myeloma?

Answer 1

• A neoplatic proliferation of Plasma cells
• neoplastic plasma cells produce Immunoglobulins
  
  • heavy chains- IgG (52%), IgA & IgM
  • Light chains- lambda & kappa
    
    • aka Bence Jones proteins
• Different forms
  
  • multiple myeloma
  • solitary plasmacytoma
  • osteosclerotic myeloma

Question 2

What is the epidemiology of multiple myeloma?

Answer 2

• Most common primary bone malignancy
• pts >40 yrs
• males>females
• prognosis variable
  
  • 5 yr survival 30%, 10 yr survival 11%
  • poor factors- chromosome 13 deletion, circulating plasma cells, increase beta 2 miroglobulin ( elevated tumour burden)

Question 3

What is the criteria for dx of multiple myeloma?

Answer 3

• One major and one minor or 3 minor fordx
• major
  
  • biopsy = plasmacytoma
  • >30% plasma cells on Bone marrow biopsy
  • serum IgG >3.5g/dl
  • urine IgA >1g/24hrs or presence of Bence Jones proteins
• Minor
  
  • 10-30% plasma cells on BM biopsy
  • serum/urine levels as above
  • multiple lytic bone lesions- punched out
  • decreased serum level of IgG

Question 4

Define solitary plasmacytoma?

Answer 4

• A solitary cell tumour occuring in a single skeletal location lacking appropriate criteria for dx of MM
• sensitive to Radiation
• progress to multiple myeloma in 50% cases
• negative BM biopsy- no plasma cells

Question 5

Define Osteoscleoritc myeloma?

Answer 5

• A rare syndrome characterised by POEMS
• Polyneuropathy- symmetrical begin distal, migrate proximally
• Organomegaly
• Endocrinopathy
• M protein
• Skin changes- occur in trunk

Question 6

What are the symptoms of multiple myeloma?

Answer 6

• Localised bone pain - usually spine/ribs
• Pathological fx
• Fatigue- 2ary to anaemia, hypercalcaemia, remal insufficiency

Question 7

What is seen on xrays of multiple myeloma?

Answer 7

• Multiple Punches out lesions
  
  • caused by osteoclastic bone resorption via RANKL, IL6 and MIP-1alpha
  • lack of osteoblastic activity- lack of scleortic border
• MRI- multiple punched out lesions, bright T2, dark T1

Question 8

What is seen on bone scans with multiple myeloma?

Answer 8

• Cold in 30%
• lacks osteoblastic activity
• so obtain skeletal survey

Question 9

What is seen on blood/urine labs in multiple myeloma?

Answer 9

• Anaemia
• elevated creatinine
• hypercalcaemia
  
  • 30% , excessive resorption of bone
• ESR elevated
• SPEP- serum protein electrophoresis
  
  • M spike present

Urine

• Proteinuria
• UPEP- urine protein electrophoresis
  
  • may show bence jones proteins- secreted kappa and lambda light chains

Question 10

What is seen on histology in multiple myeloma?

Answer 10

• Round plasma cells w eccentric nucleus
• clock face organisation of chromatin
• immuno stains CD38+

Question 11

What is the tx of multiple myeloma?

Answer 11

Non operative

• Multiagent Chemotherapy
  
  • mainstay of tx
• Bisphonates
  
  • help reduce no of skeletal events in multiple myeloma pts

Operative

• Surgical stabilisation/ irradiation
  
  • incomplete/ pending fx

Question 12

What is the tx for solitary plasmacytomas?

Answer 12

• External beam irradiation alone 45-50 Gy
  
  • mainstay of tx

Surgery

• Surgical Stabilisation
  
  • complete/ pending fx

Question 13

What is the tx for osteosclerotic myeloma?

Answer 13

• Chemotherapy, radiotherapy & plasmapheresis
  
  • mainstay of tx
  • neurology don’t improve with tx

Question 14

What is lymphoma?

Answer 14

• A maligant hamepoietic tumour uncommonly found primarily in bone that occurs in 3 forms
  
  • Primary Lymphoma of bone ( solitary site)
    
    • non Hogkins’s B cell lymphoma cf T cell
    • dx when only a single node for 6 months
  • Multiple bone sites ( no visceral sites)
  • **Bone & soft tissue lymphoma **

Question 15

What is the epidemiology of lymphoma?

Answer 15

• 10-35% of non hogkins lymphomas have extranodal disease
• primary lymphoma of bone is very rare
• males> females
• all age groups, common 35-55 yrs
• sites- pelvis. spine, ribs, knee ( distal femur/prox tibia) shoulder girdle
• Risk factirs- HIV, viral/bacterial infections
• prognosis- primary lymphoma of bone has better prognosis than secondary involvement of bone

Question 16

What are the symptoms of lymphoma?

Answer 16

• Pain- unrelived by rest
• Pathological fx- 25%
• Fever, night sweats, weight loss- B symptoms
• Neurological symtpoms from cord compression

Warm, swollen large soft tissue masses are common

Question 17

What is seen on radiographs with lymphoma?

Answer 17

• Large ill defined lytic lesions w a subtle mottled appearance
• more common in diaphysis of long bone
• 25% cortical thicknening
• multiple sites
• DDX
  
  • multiple myeloma
  • metastatic disease
  • osteomyelitis
• Ct chest, abdo & pelvis and Bone marrow aspirate for staging
• Bone scan positive

Question 18

What is seen in histology for lymphoma?

Answer 18

• not needle biopsy as tissue crushed
• mixed small round blue cells infiltrate
• diffuse infiltrations of trabeculae
• immuno stains positive for CD20, CD45, lymphocyte common antigen positive

Question 19

What is the tx for lymphoma?

Answer 19

Non operative

• Multiagent chemotherapy +/- local irradiation
  
  • mainstay of tx
  • cyclophosphamide, doxorubicin, prednislone & vincristine= CDPV
  • Chemo alone is effective in most cases
  • 70% survival at 5 yrs for disseminated disease

Operative

• Fracture stabilisation

Question 20

what is leukaemia?

Answer 20

• most common malignancy of childhood
• forms include
• ALL- acute lymphoblastic leukaemia
• peak incidence at 4yrs
• causes demeralization of bones, periostitis and lytic lesions
• ** tx = chemotherapy - may predispose to fx**