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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
How is lymphoma staged?
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
25
Very rough 5 yr survival for low and high grade nh lyphoma
Low - 80-90% | High 50-60%
26
Rough 5 year survival for h lymphoma
90%
27
What drug can be used to augment chemo in lymphoma?
Steroids
28
What notation should be added to lymphoma staging if present
b for b symptoms
29
Long term complications of treated h lymphoma
Decreased immune function | Risk of relapse and other cancers
30
Presentations of leukaemia generally
Systemic - fever, malaise Bone marrow infiltration - anaemia, infection, bleeding Organ involvement - lymphadenopathy, hepatosplenomegaly
31
Where should be checked for purpuria especially?
Pallet and retina
32
Common age effected by AML
60s
33
Risk factors for AML
``` Myelodysplastic or myeloproliferative disorders Previous chemo Viral infections Radiation exposure Chemical exposure ```
34
Prognostic indicators in AML
Cytogenetics | Poorer if background myeloproliferative or dysplastic syndromes
35
Cytogenetic variant of AML which lowers prognosis and can present as a haematological emergency?
Acute promyelocytic leukaemia | Can present with DIC due to production of thromboplastin
36
Average 5 yr survival of AML? Effect of age on this value?
27% | Much better prognosis in the young
37
Age of presentation of CML
50/60s
38
Cytogenetic abnormality associated with CML and treatment if present
Philidelphia chromosome | Imatinib, a tyrosine kinase inhibitor acting against effects of BRC:ABL
39
Typical presentation feature of CML
Massive hepato or splenomegally | Can cause splenic infarction
40
What is the effect of chemo on CML
Symptom relief but no change in prognosis
41
Other than imatinib what drug can be considered in cml?
Hydroxyurea
42
Median survival for CML? Improved markedly by what?
3-4 years | If imatinib sensitive 70% 5yr survival
43
Common age of presentation of ALL
5-7
44
What lineage of cells typically causes ALL. What is different about epidemilogy of other liniage
B | T cell ALL tends to effect adolescents
45
Complication that effects 75% of ALL patients if not treated. Requires special chemotherapy
Meningeal leukaemia Rasied icp and cn palsys Needs intrathecal chemotherapy. Check CSF early
46
Prognosis of ALL. What worsens it?
80% cure | Decreases with old age and t cell disease
47
Typical presentation of CLL
Often incidental May give lymphadenopathy, splenomegally etc. Idolent course, bone marrow failure a late symptom
48
What treatment should be given to indolent CLL
Consider antibitoics, antifungals and antivirals prophylactically
49
If more agressive CLL what treatments to consider
Steroids, chemo radio
50
Commonest paraproteins produced by myeloma
IgG
51
What is the term for localised myeloma? What is it amenable too?
Solitary plasmacytoma | Radiotherapy
52
Main symptom groups of myeloma
Bone marrow invasion Bone erosion Paraprotein production
53
Signs and symptoms of bone erosion in myeloma
Pain Pathalogical fracture Vertebral collapse Hypercalcaemia
54
Signs and symptoms of paraprotein production in myeloma
Hyperviscocisy - neurological findings Renal failure/aki Immune paresis - infection Amyloid deposition - carpal tunnel syndrome, diarrheoa, macroglossia
55
Abnormal bloods in myeloma
Raised esr Bone marrow failure picture Renal failure picture Hypercalcaemia
56
Other than bloods investigations in myeloma
Xray/skeletal survey - lytic lesion | Serum/urine electrophoresis
57
Treatments of myeloma
Supportive - pain relief, transfusions, epo, radiotherapy, bisphosphonates, abx Specific - stem cell transplant, steroids, chemo, thalidomide
58
Prognosis of myeloma
Median survival 36 months
59
Differential diagnosis of myeloma? differentiate!
Monoclonal gammopathy of uncertain significance | - no bence jones proteins,
60
Prevalence of MGUS in elderly | Prognosis of. MGUS
3 % | 25 % develop myeloma or al amyloidosis
61
Treatment of essential thrombocythaemia
Hydroxyurea
62
Pathology of myelofibrosis
Proliferation of megakaryocytes and granulocytes leading to growth factor (pdgf) release and fibrosis of bone marrow. Causes extramedullary haemoptysis.
63
Presentation of myelofibrosis
Splenomegaly, hepatomegaly, lymphadenopathy | Night sweats, weight loss, fever
64
Prognosis of myelofibrosis
Median survival 3-5 yrs due to bone marrow failure | 10% convert to AML
65
Treatment options of myelofibrosis
Symptomatically - transfusions - hydroxycarbomide

FPE (11 decks)

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