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Flashcards in Haematopoietic Deck (20):
1

Define mutliple myeloma?

  • 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

2

What is the epidemiology of multiple myeloma?

  • 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)

3

What is the criteria for dx of multiple myeloma?

  • 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

4

Define solitary plasmacytoma?

  • 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

5

Define Osteoscleoritc myeloma?

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

6

What are the symptoms of multiple myeloma?

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

7

What is seen on xrays of multiple myeloma?

  • 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

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8

What is seen on bone scans with multiple myeloma?

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

9

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

  • 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

10

What is seen on histology in multiple myeloma?

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

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11

What is the tx of multiple myeloma?

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

12

What is the tx for solitary plasmacytomas?

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

Surgery

  • Surgical Stabilisation
    • complete/ pending fx

13

What is the tx for osteosclerotic myeloma?

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

14

What is lymphoma?

  • 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 

15

What is the epidemiology of lymphoma?

  • 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

16

What are the symptoms of lymphoma?

  • 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

17

What is seen on radiographs with lymphoma?

  • 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

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18

What is seen in histology for lymphoma?

  • 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 

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19

What is the tx for lymphoma?

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

20

what is leukaemia?

  • 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