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1MRCP Part 1 > Haematology and Oncology > Flashcards

Haematology and Oncology Flashcards

1
Q

Blood Film Findings in Hyposplenism

A

Howell Jolly Bodies

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2
Q

Lemiere’s Sydrome

A

= thrombophelbitis of internal jugular vein
Usually occurs as secondary to throat infection

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3
Q

Disproportionate Microcytosis in Anaemia

A

Thalassaemia Trait

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4
Q

Mild Haemophilia A
- Needs procedure e.g. dental work - what is given?

A

Desmopressin
= promotes endogenous factor VIII and vWF factor production to avoid need for exogenous

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5
Q

Management of benefit in acute chest crisis
Transfusion threshold

A

Incentive spirometry
Usually >100 aiming for Hb - haematologist decision

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6
Q

Myelofibrosis blood film finding

A

Tear drop poikilocytes
- shedding a tear because fear of myelofibrosis

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7
Q

CD marker found on Reed Sternberg cells

A

CD 15

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8
Q

Leukaemoid reaction

A

Left shift neutrophils
= bone marrow reaction to stress or infection

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9
Q

Management of Acute Promyelocytic Leukaemia

A

All-trans retinoic acid (ATRA)

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10
Q

Pathogenesis of TTP

A

Failure to cleave vWF normally
- deficiency of ADAMTS13 protein which usually does the cleaving

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11
Q
A
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11
Q

What is LAP?
High LAP
Low LAP

A

LAP = leukocyte alkaline phosphatase
Found within mature WBC
High = mature WBC pathology e.g. myelofibrosis
Low = immature WBC pathology e.g. CML

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12
Q

What is associated with a poor prognosis in lymphoma?

A

B symptoms

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13
Q

Mechanism of action of LMWH heparin

A

Activation of antithrombin III
Inhibits factor Xa only
(LMWH is low-key, activates AT3)

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13
Q

Mechanism of action of standard heparin

A

Inhibits factors inc Xa, IXa, Xia, XIIa

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14
Q

Mechanism of action of DOAC

A

Direct Xa inhibition

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15
Q

Complication of plasma exchange
- why?

A

Hypocalcaemia
Contains sodium citrate which chelates calcium
Citrate used as anticoagulant

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16
Q

Test for hereditary spherocytosis

A

EMA binding test
- has replaced osmolality fragility test

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17
Q

Types of Hb

A

A - adult (a+b chains)
A2
Foetal (a+gamma chains)

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18
Q

Iron found in
- haemoglobin
- myoglobin
- Methahaemoglobin

A

Hb = Fe2+
Myo = Fe2+
Metha = Fe3+

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19
Q

Oxygen Dissociation Curve
- shift to the right =
- shift to the left =

A

Right = reduced affinity for oxygen, releases O2 at tissues better

Left = increased affinity for oxygen, doesn’t release as easily (sticky)

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20
Q

Factors which shift the Hb curve to the left (5)

A

= increased affinity for O2
LOW pCO2
LOW H+ (alkali)
LOW temp
HIGH carbon monoxide
Foetal haemoglobin

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21
Q

Factors which shift the Hb curve to the right (3)

A

= decreased affinity for O2
HIGH H+ (acid)
HIGH temp
HIGH pCO2

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22
Q

Pathophysiology of sickle cell anaemia
- Homozygous
- Heterozygous

A

Amino acid substitution on B chain
Can only make A2 and F haemoglobin if homozygous
Can make HbA if heterozygous

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23
Why is haptoglobin low in haemolytic anaemia?
Haptoglobin binds to free Hb and clears it = consumed in haemolytic anaemia = low
24
What is the cause of factor V leiden?
Activated protein C resistance (HOT TO GO)
25
In ITP what are the antibodies directed against?
Glycoprotein
26
Herceptin =
Trastuzumab Used in HER2 positive breast cancer`
27
Pathophysiology of CLL
= monoclonal B cell proliferation (vs myeloma which is plasma cell clones)
28
Positive diagnosis of anti-phospholipid syndrome
Antibodies e.g. anti-cardiolipin, glycoprotein B2 on at least 2 occasions, 12 weeks apart
29
AA vs AL amyloid `
AA = inflammatory conditions e.g. rheumatoid arthritis, renal presentation most common AL = more common, 2y to malignancy
30
Chromosome associated with alpha thalassaemia
Ch 16 (almost 16 letters)
31
Why do you give desmopressin in vWF
Desmopressin encourages release of vWF from the endothelial cells (similar mechanism to use in haemophilia)
32
t11:14 association
Mantle cell lymphoma
33
What is the mechanism of action in Heparin induced thrombocytopaenia?
Antibodies against complexes of platelet factor and heparin
34
What is used as prophylaxis in sickle cell disease?
Hydroxyurea
35
Gene mutation in hairy cell leukaemia
BRAF
36
Mastocytosis VS Mast cell leukaemia
No blasts on blood film
37
Why is INF-a useful in hairy cell leukaemia?
INFa = anti-viral action Useful in malignancies associated with viruses e.g. kaposi's sarcoma, hairy cell leukaemia
38
Presentation of hairy cell leukaemia (4)
Splenomegaly Weight loss Lymphocytosis Cells with broad based projections
39
Vincristine - mechanism - side effect
Inhibits mitosis S/E: peripheral neuropathy
40
Cyclophosphamide - mechanism - side effect
Promotes cross linking of DNA S/E: haemorrhagic cystitis
41
Doxorubicin - mechanism - side effect
Stabilises DNA topoisomerase which inhibits DNA/RNA synthesis S/E: cardiomyopathy
42
Bleomycin - mechanism - side effect
Degrades DNA S/E: lung fibrosis - lower zones
43
Cisplatin - side effects (4)
Nephrotoxic Ototoxic Peripheral neuropathy Hypomagnesaemia
43
Causes of intravascular haemolysis (5)
Acute haemolytic transfusion reaction G6PD deficiency Cold AIHA Paroxysmal nocturnal haemoglobinuria Red cell fragmentation
44
Extravascular causes of haemolysis (4)
Sickle cell disease Thalassaemia Haemolytic disease of the newborn Warm AIHA
44
What is the cause of a positive Schumm test?
Intravascular haemolysis
45
Causes of cold AIHA (3)
Mycoplasma EBV Lymphoma
46
Causes of warm AIHA (4)
Penicillin Cephalosporins Lymphoma SLE
47
Ch responsible for alpha chain in Hb
Ch 16
48
Genetic issue in sickle cell disease
Amino acid substitution Glutamate substitutes for valine at codon 6
49
Ch responsible for beta chain Hb
Ch 11
50
Lead poisoning - microscope finding - defect
Basophilic stippling Defect in ferrochelatase ALA dehydratase
51
Defect in sideroblastic anaemia - finding
Delta aminolevulinate synthase 2 Sideroblasts
52
What is the role of G6PD?
Promotes production of NADPH which promotes generation of gluthianone which protects RBC from oxidative damage
53
Pathophysiology of paroxysmal nocturnal haemoglobinuria
Pig A mutation Reduced GPI Reduced DAF - results in unregulated complement damage and intravascular AIHA
54
Investigation of paroxysmal nocturnal haemoglobinuria
Flow cytometry - see a lack of CD59/CD55
55
What is the indirect coomb's test used for?
Detects free and unbound antibodies against specific antigens
56
Worst prognosis of Hodgkin's lymphoma
Lymphocyte depleted
57
What is the Schumm test?
When haptoglobin becomes saturated the Hb binds to albumin = methemalbumin
58
What is the most common subtype of Hodgkin's Lymphoma?
Nodular sclerosing
59
Genetic mutation associated with Burkitt's lymphoma
c-myc 8:14 translocation
60
What is a stage 3 Hodgkin's lymphoma?
= positive lymph nodes either side of the diaphragm
61
What is JAK2?
It is a non-receptor tyrosine kinase which stimulates growth
61
Percentage with myelodysplasia which progress to AML?
30%
62
Why do you get splenomegaly in myelofibrosis? - investigation finding
Bone marrow fibrosis means there is extramedullary haematopoiesis Tear drop poikilocytes
63
Primary polycythaemia =
= polycythaemia rubra vera
64
Investigation findings in PRV (4)
Low ESR High leukocyte ALP High Hb Low epo
65
Management of essential thrombocytosis
Hydroxyurea
66
Features suggestive of poor prognosis in AML (2)
<60 years Deletions at Ch 5 or 7
67
Investigation findings in AML (3)
Auer rods >30% blasts in BM 15:17 in APML
68
Induction chemotherapy in AML
Cytarabine + anthracycline (then consolidation)
69
Management of CML (2)
Imatinib Hydroxyurea
70
Investigation findings in CML (3)
Ch 9: 22 - philadelphia chromosome = BCR-ABL proto-oncogene Low leukocyte ALP
71
Poor prognostic markers in CLL (2) Association with CLL
TP53 CD38 Warm AIHA
71
Richter's transformation ?
Transformation of CLL to high grade NHL
72
Management of CLL - chemotherapy regimen
RT usually Chemo = FCR Fludarabine, Cyclophosphamide, Rituximab
72
Good prognosis in CLL
13q14 deletion
73
Poor prognostic markers in ALL (2)
4:11 translocation Philadelphia chromosome
73
Features of hyposplenism on blood film (3)
Howell Jolly bodies Target cells Pappenheimer bodies
74
Features of IDA on blood film (2)
Target cells Pencil poikilocytes
75
Feature of megaloblastic anaemia on blood film
Hypersegmented neutrophils
76
What is hairy cell leukaemia?
A subset of CLL
77
Association of AL amyloidosis
Malignancy - MGUS, waldenstrom's, myeloma
78
Association of AA amyloid (3)
Chronic infection SLE TB
79
79
What is Waldenstrom's macroglobulinaemia associated with?
Cryoglobulinaemia I (monoclonal proliferation)
80
mGUS vs myeloma investigation finding
mGUS = normal B2 microglobulin
81
Causes of acquired Haemophilia A (6) - mechanism
Psoriasis Malignancy Pemphigus IBD Phenytoin RA = IgG antibodies with factor VIII
81
Mechanism of action of TXA
Inhibits plasminogen conversion to plasmin
82
Investigation findings in vWF (2)
Slightly raised APTT Reduced factor VII
83
Primary haemostasis - 3 steps
Platelet adhesion - via GP1b receptors and vWF Platelet activation - release of ADP to GP2b/3a receptors and release of TA2 resulting in chemotaxis Platelet aggregation = platelet plug
84
Problem in methahaemaglobinaemia
Fe2+ oxidised to Fe3+ = reduced capacity for O2 carrying, high affinity for oxygen, poor release of O2 at tissues
85
Investigation finding in methahaemoglobinaemia Management
ABG - normal PaO2 but low SaO2 Methylene blue
86
What is the mechanism in ITP?
IgG antibodies against GP2b3a
87
What is Evan's syndrome?
ITP + AIHA
88
Features of systemic mastocytosis (3)
Urticaria Flushing Darrier's sign = wheals appearing on rubbing the skin
88
Management of HIT?
Stop heparin Do not give warfarin - can trigger necrosis skin reaction
89
Genetic change in haemochromatosis
HFE gene on chromosome 6
90
What can you reverse in haemochromatosis with treatment?
Skin pigmentation DCM
91
What do you need to do prior to elective splenectomy?
Vaccinations 2/52 before Pneumococcus Haemophilus influenza Men A/C Influenza
92
Investigation findings in haemochromatosis (3)
Raised transferrin Raised ferritin Chondrocalcinosis

1MRCP Part 1 (20 decks)

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