Haem Cancers Flashcards

1
Q

What are the main myeloproliferative disorders/neoplasms?

A

Chronic myelogenous leukaemia
Polycythemia vera
Essential thrombocythaemia
Primary myelofibrosis

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2
Q

What is the potential course of myeloproliferative disorders/neoplasms?

A

Evolution into another myeloproliferative disorder or acute leukaemia

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3
Q

What genes tend to be effected in the myleoproliferative disorders/neoplasms?

A

CML - bcr-abl (philidelphia chromosome)

PV, ET, MF - JAK2

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4
Q

Broad categorisation and causes of pooycythemia

A

Relative - decreased plasma volume
Absolute:
1) Primary - polycythemia vera
2) Secondary - altitude, smoking, hypoxic lung diseases, iatrogenic

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5
Q

Signs and symptoms of polycythaemia vera

A
Headache, pruritus, blurred vision, night sweats
Plethoric 
Splenomegally
Haemorrhage
Thrombosis (MI, DVT, PE, CVA)
HTN
Gout
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5
Q

How can primary and secondary absolute polycythemia be differentiated with bloods?

A

Look at EPO, in secondary will be high, in primary will be supressed
Look for JAK2 mutation

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6
Q

What is treatment of polycythemia vera aimed at avoiding? What does it not effect? Precentage effected by such complications

A

Reduces vte risk (20%)

Doesnt effect transformation to acute leukaemia (10%) or fibrosis (5%)

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7
Q

Treatment of all polycythemia vera patients

A

Phlebotomy
Daily aspirin
Consideration to hydroxycarbamide

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8
Q

What are the b symptoms of lymphoma?

A

Fever
Night sweats
Weight loss

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9
Q

Additional non-B systemic symptoms of lymphoma

A
Pruritus
Lethargy 
Loss of appetite 
Bone marrow infiltration with anaemia, thrombocytopenia and infections 
Liver/spleen/tonsils enlargement
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10
Q

Age ranges vulnerable to hodgekins and non-hodgekins lymphoma?

A

H - 20s and >60s

NH - >60s

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11
Q

Non infectious PMH that specifically raises risk of non-hodgekins lymphoma

A

Coeliac

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12
Q

Infections that raise risk of lymphoma of both types

A

HIV

EBV

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13
Q

Name of non-hodgkins lymphoma caused by EBV?

A

Burkitts

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14
Q

What specific symptoms point to hodgkins over non hodgekins lymphoma?

A

Alcohol induced lymph node pain

Pal ebstein fever - cyclical over 1-2 weeks

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15
Q

What effects can lymphoma cause locally to enlarged nodes?

A
Neuropathies
Breathlessness (compressing airways) 
Lymphoedema 
Jaudice (blocking bile duct) 
Neurological (in the brain)
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16
Q

What differentiates hodgkins from non hodgekins lymphoma? Histological features?

A

Reed sternberg cells
Bi/multinucleated large b cell derivitives
Owl eye apperence

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17
Q

What blood tests should follow a dx of lymphoma?

A

FBC
ESR
LDH

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18
Q

What should be screened for following Dx of lymphoma?

A

HBV HIV

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19
Q

Purpose of albumin and LDH testing in lymphoma?

A

Prognostic

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20
Q

Types of hodgkins lymphoma? Common to rare

A
Classical
-nodular sclerosing  
-lymphocyte rich
-mixed cellularity 
-lymphocyte deplete
Nodular lymphocyte predominant
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21
Q

How can non hodgkins lymphoma be subclassified broadly?

A

Low vs high grade
B vs T
Large or small cell
Follicular or diffuse

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22
Q

Does subtype of lymphoma have more effect in H or NH?

A

H

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23
Q

What unusual types of NH lymphoma are there?

A

Skin lymphomas
MALT lymphomas
Mantle cell

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24
Q

How is lymphoma staged?

A

Ann Arbour
1 - one group of nodes or lymphoid organ
2 - two or more groups one side of diaphragm
3 - nodal groups both sides of diaphragm
4 - into non lymphoid organ

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25
Q

Very rough 5 yr survival for low and high grade nh lyphoma

A

Low - 80-90%

High 50-60%

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26
Q

Rough 5 year survival for h lymphoma

A

90%

27
Q

What drug can be used to augment chemo in lymphoma?

A

Steroids

28
Q

What notation should be added to lymphoma staging if present

A

b for b symptoms

29
Q

Long term complications of treated h lymphoma

A

Decreased immune function

Risk of relapse and other cancers

30
Q

Presentations of leukaemia generally

A

Systemic - fever, malaise
Bone marrow infiltration - anaemia, infection, bleeding
Organ involvement - lymphadenopathy, hepatosplenomegaly

31
Q

Where should be checked for purpuria especially?

A

Pallet and retina

32
Q

Common age effected by AML

A

60s

33
Q

Risk factors for AML

A
Myelodysplastic or myeloproliferative disorders
Previous chemo
Viral infections 
Radiation exposure
Chemical exposure
34
Q

Prognostic indicators in AML

A

Cytogenetics

Poorer if background myeloproliferative or dysplastic syndromes

35
Q

Cytogenetic variant of AML which lowers prognosis and can present as a haematological emergency?

A

Acute promyelocytic leukaemia

Can present with DIC due to production of thromboplastin

36
Q

Average 5 yr survival of AML? Effect of age on this value?

A

27%

Much better prognosis in the young

37
Q

Age of presentation of CML

A

50/60s

38
Q

Cytogenetic abnormality associated with CML and treatment if present

A

Philidelphia chromosome

Imatinib, a tyrosine kinase inhibitor acting against effects of BRC:ABL

39
Q

Typical presentation feature of CML

A

Massive hepato or splenomegally

Can cause splenic infarction

40
Q

What is the effect of chemo on CML

A

Symptom relief but no change in prognosis

41
Q

Other than imatinib what drug can be considered in cml?

A

Hydroxyurea

42
Q

Median survival for CML? Improved markedly by what?

A

3-4 years

If imatinib sensitive 70% 5yr survival

43
Q

Common age of presentation of ALL

A

5-7

44
Q

What lineage of cells typically causes ALL. What is different about epidemilogy of other liniage

A

B

T cell ALL tends to effect adolescents

45
Q

Complication that effects 75% of ALL patients if not treated. Requires special chemotherapy

A

Meningeal leukaemia
Rasied icp and cn palsys
Needs intrathecal chemotherapy. Check CSF early

46
Q

Prognosis of ALL. What worsens it?

A

80% cure

Decreases with old age and t cell disease

47
Q

Typical presentation of CLL

A

Often incidental
May give lymphadenopathy, splenomegally etc.
Idolent course, bone marrow failure a late symptom

48
Q

What treatment should be given to indolent CLL

A

Consider antibitoics, antifungals and antivirals prophylactically

49
Q

If more agressive CLL what treatments to consider

A

Steroids, chemo radio

50
Q

Commonest paraproteins produced by myeloma

A

IgG

51
Q

What is the term for localised myeloma? What is it amenable too?

A

Solitary plasmacytoma

Radiotherapy

52
Q

Main symptom groups of myeloma

A

Bone marrow invasion
Bone erosion
Paraprotein production

53
Q

Signs and symptoms of bone erosion in myeloma

A

Pain
Pathalogical fracture
Vertebral collapse
Hypercalcaemia

54
Q

Signs and symptoms of paraprotein production in myeloma

A

Hyperviscocisy - neurological findings
Renal failure/aki
Immune paresis - infection
Amyloid deposition - carpal tunnel syndrome, diarrheoa, macroglossia

55
Q

Abnormal bloods in myeloma

A

Raised esr
Bone marrow failure picture
Renal failure picture
Hypercalcaemia

56
Q

Other than bloods investigations in myeloma

A

Xray/skeletal survey - lytic lesion

Serum/urine electrophoresis

57
Q

Treatments of myeloma

A

Supportive
- pain relief, transfusions, epo, radiotherapy, bisphosphonates, abx
Specific
- stem cell transplant, steroids, chemo, thalidomide

58
Q

Prognosis of myeloma

A

Median survival 36 months

59
Q

Differential diagnosis of myeloma? differentiate!

A

Monoclonal gammopathy of uncertain significance

- no bence jones proteins,

60
Q

Prevalence of MGUS in elderly

Prognosis of. MGUS

A

3 %

25 % develop myeloma or al amyloidosis

61
Q

Treatment of essential thrombocythaemia

A

Hydroxyurea

62
Q

Pathology of myelofibrosis

A

Proliferation of megakaryocytes and granulocytes leading to growth factor (pdgf) release and fibrosis of bone marrow. Causes extramedullary haemoptysis.

63
Q

Presentation of myelofibrosis

A

Splenomegaly, hepatomegaly, lymphadenopathy

Night sweats, weight loss, fever

64
Q

Prognosis of myelofibrosis

A

Median survival 3-5 yrs due to bone marrow failure

10% convert to AML

65
Q

Treatment options of myelofibrosis

A

Symptomatically

  • transfusions
  • hydroxycarbomide