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Tan Haematology > Malignancy > Flashcards

Malignancy Flashcards

1
Q

Myeloma definition

A

Malignant proliferation of plasma cells in the bone marrow characterised by

  1. a monoclonal paraprotein in the serum/urine
  2. bone changes leading to pain and pathological fractures
  3. excess plasma cells in the bone marrow
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2
Q

features of myeloma

A
  • median age 60-65 years
  • CRAB
  • renal failure
  • hypercalcaemia
  • anaemia
  • infections
  • spinal cord compression (plasmacytomas), general bone pain
  • amyloidosis (–> macroglossia, hepatosplenomegaly, cardiac failure, carpal tunnel syndrome and autonomic neuropathy
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3
Q

bone destruction in myeloma

A
  • lytic lesions on xray
  • osteoporosis AND lytic lesions
  • vertebral collapse –> loss of height, back pain, kyphosis
  • “pathological” fracture
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4
Q

Laboratory features of myeloma

A
  • Anaemia (often with neutropenia, thrombocytopenia and ESR >100)
  • Rouleaux on blood film with bluish background from increased protein
  • Bone marrow >10% plasma cells often w/ multinucleate
  • Paraprotein in serum and/or Bence Jones proteins in urine with suppression of normal serum Igs (can be IgG, IgA, IgM uncommon)
  • Raised serum ß2 microglobulin often
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5
Q

Treatment of myeloma

A

Observation for symptomless patients (no CRAB)

If symptomatic
>70 years –> chemotherapy e.g. thalidomide, lenalidomide, bortezomib

<70 years –> chemotherapy e.g. melphalan + high dose therapy with autologous stem cell transplant

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6
Q

Mechanism of myeloma bone symptoms

A

Myeloma cells produce factors that result in:

  1. Activation of osteoclasts e.g. RANKL
  2. Inhibition of osteoblasts e.g. DKKI

These lead to bone resorption

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7
Q

Pain management in myeloma

A
  • analgesics (caution in renal impairment)
  • chemotherapy
  • bisphosphonates
  • radiotherapy (localised, severe pain)
  • orthopaedic surgery –> fixation
  • general measures - mobility, physio
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8
Q

Other causes of paraproteinaemia?

A

Other B cell or plasma cell neoplasms

  • MGUS
  • plasmacytomas
  • Primary amyloidosis
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9
Q

What is MGUS?

A

Monclonal Gammopathy of Undetermined Significance

  • Incidence increases from 5th decade onwards
  • 1% per year evolve to myeloma, then watch and wait
  • paraprotein present but less than myeloma levels
  • bone marrow plasma cells <10%
  • NO lytic lesions
  • no myeloma-related symptoms
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10
Q

When to suspect myeloma?

A

Paraprotein +

  • *C**alcium elevated
  • *R**enal impairment
  • *A**naemia
  • *B**one pain/lytic lesions
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11
Q

What is a plasmacytoma?

A

Clonal proliferation of plasma cells identical to those in myeloma, but manifest as localised mass in bone or soft tissue:

  • solitary plasmacytoma of bone
  • solitary extramedullary plasmacytoma

Treatment: radiotherapy if truly localised, ie no underlying myeloma

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12
Q

What is primary amyloidosis (aka systemic AL)

A
  • Protein confirmation disoreder associated with a clonal plasma cell problem, like a form of light chain MGUS
  • multiple organ disease occurs from extracellular deposition of insoluble light chain fragments
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13
Q

How does Primary Amyloidosis affect different organs?

A
  • Heart - congestive cardiomyopathy
  • Kidneys - nephrotic syndrome +- renal insufficiency
  • Nerves - peripheral neuropathy
  • Liver - hepatomegaly
  • Gut - macroglossia, malabsorption
  • Skin..
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14
Q

How do you diagnose primary amyloidosis?

A

Tissue biopsy of affected organ + SC fat aspirate

Also assess plasma cells abnormality: serum/urine electrophoresis BM biopsy + skeletal survey

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15
Q

Treatment of primary amyloidosis?

A
  • Organ specific - supportive treatments
  • chemotherapy similar to myeloma (treats plasma clone not amyloid)
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16
Q

Difference between leukaemia and lymphoma

A

The distinction may be blurred
Leukaemia

  • Widespread involvement of bone marrow
  • tumour cells in peripheral blood

Lymphoma

  • Discrete tumour masses
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17
Q

What is lymphoma

A

Malignant proliferation of lymphocytes. Derived from a single transformed cell

  • monoclonal
  • same antigen receptor gene rearrangement
  • Reside where normal counterparts found
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18
Q

Types of lymphoma

A
  • Hodgkin’s Lymphoma
    • may be localised or widespread
  • Non Hodgkin’s
    • Usually disseminated at diagnosis
      • B cell
      • T cell and NK cell
      • Histiocytic and dendritic cell
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19
Q

What is Hodgkin’s Lymphoma

A
  • Malignant disorders of lymphoid tissue
  • Reed-Sternberg and mononuclear Hodgkin’s cells form a minority of the tumour
  • Rest from lymphoblasts, granulocytes, fibroblasts and plasma cells
  • EBV may play a role
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20
Q

AML causes what blood results?

A
  • Anaemia
  • Neutropenia
  • Thrombocytopenia

therefore symptoms of anaemia, infection and haemorrhage

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21
Q

AML diagnostic tests

A
  1. Blood count and film - elevated WCC, can be normal/low, often blast cells, auer rods
  2. Bone marrow aspirate and trephine - blast infiltration
  3. Cytochemistry - positivity with Sudan black and myeloperoxidase
  4. Immunophenotyping - myeloid antigens e.g. CD13 and CD33
  5. Cytogenetics - BM sample sent
  6. Molecular Biology - PCR, FISH
22
Q

AML treatment

A
  • Red cell transfusion
  • Platelet concentrates
  • Broad spectrum Abx
  • Chemotherapy -
    • aiming for <5% blast cells in BM (complete remission)
    • induction then consolidation
    • daunorubicin and arabinoside
  • Autologous stem cell transplantation
23
Q

AML characteristic blood film features?

A

Auer rod in blast cells

24
Q

AML definition

A

Presence of >20% blast cells in the BM at clinical presentation

25
Q

AML presentation

A
  • More common in the elderly
  • Linked to Down’s syndrome and myelodysplasia
  • Gum hypertrophy and infiltration
  • CNS and skin involvement
  • Bleeding
  • common genetic mutation ASXL1
26
Q

ALL pathophysiology

A

Malignancy of lymphoid cells can affect B and T cell lineages (more commonly B)

arrest maturation and promote proliferation of immature blasts

27
Q

ALL presentation

A
  • 2 peaks at 3-7 years and over 40yo
  • Anaemia, bleeding, infection (BM failure)
  • Hepatosplenomegaly
  • Testicular infiltration
  • Lymphadenopathy (parotid)
  • CNS involvement
  • B symptoms
  • Associated with Down’s and radiation during pregnancy
  • FAB classification (L1, L2, L3)
28
Q

ALL treatment

A
  • Chemotherapy
    • vincristine, prednisolone, asparaginase, maybe MTX, cyclophosphamide and cytosine arabinoside
  • Allogenic SCT in young patients
  • Neutropenic regimen
  • Prophylactic Abx

** can get tumour lysis syndrome - give allopurinol

29
Q

ALL investigations

A
  • >20% blasts in BM
  • Pancytopenia
  • Lumbar puncture
30
Q

What is CML?

A
  • Clonal myeloproliferative disorder which results from an acquired change in a pluripotent stem cell
  • –> gross overproduction of neutrophils and their precursors
  • Three phases
    • chronic phase (benign)
    • accelerated phase
    • blast crisis (fatal)
31
Q

Genetics of CML?

A
  • Philadelphia Chromosome!
  • Translocation of 9 and 22
  • Causes BCR-ABL gene which encodes a protein with high tyrosine kinase activity
32
Q

CML presentation

A
  • Usually present in chronic phase
  • anaemia, anorexia, weight loss, bruising
  • Splenomegaly - pain, bloating, satiety
  • Occasionally gout or hyperviscosity
  • 40 - 60 years, M>F
  • blast crisis - typical acute leukaemia symptoms
33
Q

CML diagnostic Ix

A
  • WCC above 100x10^9
  • increase in myeloid cells, but highest numbers of myelocytes and neutrophils
  • often absolute basophilia
  • thrombocytosis and nucleated red cells may be present
  • Increased urate
  • FISH
34
Q

CML treatment

A
  • TKI - imatinib, dasotinib
  • chemo
  • allogenic SCT

hydroxycarbamide can be used to rapidly reduce WCC

35
Q

What is CLL

A
  • Clonal proliferation of B-lymphocytes
  • Commonest leukaemia in western world
  • Disease of the elderly, M>F
36
Q

CLL presentation

A
  • Lymphadenopathy
  • splenomegaly
  • immunosuppression/recurrent infections
  • anaemia
  • B symptoms
  • can be asymptomatic

risk of herpes zoster, other infections, autoimmune haemolysis

37
Q

CLL diagnostic Ix

A
  • High lymphocyte count confirmed by blood film
  • Cells resemble normal mature lymphocytes but often slightly larger with tendancy to burst - smear cells
  • In situ or flow cytometry techniques to identify characteristics and single Ig lightchain
  • BM aspirate shows increased numbers of small lymphocytes
  • blood film may suggest autoimmune haemolysis or autoimmune thrombocytopenia
  • *B DISEASE**
    • B lymphocytes (95%)
    • Bone marrow failure
    • Bleeding
    • Broken cells (smear cells)
38
Q

CLL staging

A

Rai and Binet:

  • Stage A: No anaemia or thrombocytopenia; less than 3 lymphoid areas enlarged
  • Stage B: No anaemia or thrombocytopenia, Three or more lymphoid areas enlarged
  • Stage C: Anaemia (Hb >100g/L) and/or plateless <100x10^9/L
    0 - 12 year survival??
39
Q

CLL Treatment

A
  • Watch and wait until symptomatic - when disease is progressing rapidly
  • RFC: rituximab, fludarabine and cyclophosphamide
    *
40
Q

Hodgkin’s presentation?

A
  • asymmetrical, painless lymphadenopathy, rubbery
  • splenomegaly/hepatosplenomegaly occur
  • B symptoms, pruritus
  • LN pain after alcohol

Emergency: SVC obstruction - raised JVP, facial oedema, SOB, blackouts

41
Q

Diagnosis of Hodgkin’s

A
  • Biopsy of lymph node, BM aspiration and trephine biopsy in advanced cases
  • whole body CT scan for staging, sypplemented by MRI and PET
  • (may have been anaemia and increased LDH)
42
Q

Hodgkin’s treatment

A
  • radiotherapy
  • chemotherapy ABVD
    • adriamycin
    • bleomycin
    • vinblastine
    • dacarbazine
43
Q

What is Non Hodgkin’s Lymphoma?

A

Solid tumours of lymphoid tissue which are not Hodgkin’s

Most common haem malignancy

Presents at any age but median age is 50

44
Q

Non Hodgkin’s Lymphoma classification

A
  • High grade
    • large poorly differentiated lymphoid cells
    • aggressive course but often curable
  • Low grade
    • smaller, better differentiated cells
    • more indolent clincally but more likely to repeatedly relapse
45
Q

Non Hodgkin’s Lymphoma presentation

A
  • More irregular spread and extranodal involvement that HL
  • Painless lymphadenopathy
  • Extranodal involvement e.g. intestinal may cause abdo pain
  • may affect CNS, may arise in skin
  • B symptoms
46
Q

Non Hodgkin’s Lymphoma diagnosis

A
  • Tissue biopsy - usually LN
  • Histology
  • Immunophenotyping - degree of maturation, B or T cell
  • Staged with CT, MRI or PET
47
Q

Treatment for Non Hodgkin’s Lymphoma

A
48
Q
  • Low grade
    • often incurable
    • Radiotherapy
    • Interferon alpha or rituximab
  • High grade
    • RCHOP
      • rituximab
      • cyclophosphamide
      • Hydroxydaunorubicin
      • Vincristine
      • Prednisolone
A
49
Q

What is aplastic anaemia?

A

Pancytopenia due to reduction in the number of pluripotent stem cells. May be exacerbated by marrow abnormality and autoimmunity

50
Q

Presentation of aplastic anaemia

A
  • Marrow failure - anaemia
  • severe infections caused by neutropenia
  • haemorrhagic tendancy - thrombocytopenia
  • may be gradual onset
51
Q

What is smouldering myeloma?

A

The transition point from MGUS to myeloma. Dx criteria include:

  • monoclonal protein in serum >= 30g/L
  • Monoclonal plasma cells >= 10% in BM or tissue biopsy
  • No evidence of end-organ damage
    • normal serum calcium, Hb, serum creatinine, no bone lesions

Should not be treated but watched closely

Tan Haematology (5 decks)

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