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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
Q

Why is haptoglobin low in haemolytic anaemia?

A

Haptoglobin binds to free Hb and clears it
= consumed in haemolytic anaemia
= low

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24
Q

What is the cause of factor V leiden?

A

Activated protein C resistance
(HOT TO GO)

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25
Q

In ITP what are the antibodies directed against?

A

Glycoprotein

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26
Q

Herceptin =

A

Trastuzumab
Used in HER2 positive breast cancer`

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27
Q

Pathophysiology of CLL

A

= monoclonal B cell proliferation
(vs myeloma which is plasma cell clones)

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28
Q

Positive diagnosis of anti-phospholipid syndrome

A

Antibodies
e.g. anti-cardiolipin, glycoprotein B2
on at least 2 occasions, 12 weeks apart

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29
Q

AA vs AL amyloid `

A

AA = inflammatory conditions e.g. rheumatoid arthritis, renal presentation most common

AL = more common, 2y to malignancy

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30
Q

Chromosome associated with alpha thalassaemia

A

Ch 16
(almost 16 letters)

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31
Q

Why do you give desmopressin in vWF

A

Desmopressin encourages release of vWF from the endothelial cells
(similar mechanism to use in haemophilia)

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32
Q

t11:14 association

A

Mantle cell lymphoma

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33
Q

What is the mechanism of action in Heparin induced thrombocytopaenia?

A

Antibodies against complexes of platelet factor and heparin

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34
Q

What is used as prophylaxis in sickle cell disease?

A

Hydroxyurea

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35
Q

Gene mutation in hairy cell leukaemia

A

BRAF

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36
Q

Mastocytosis VS Mast cell leukaemia

A

No blasts on blood film

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37
Q

Why is INF-a useful in hairy cell leukaemia?

A

INFa = anti-viral action
Useful in malignancies associated with viruses
e.g. kaposi’s sarcoma, hairy cell leukaemia

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38
Q

Presentation of hairy cell leukaemia (4)

A

Splenomegaly
Weight loss
Lymphocytosis
Cells with broad based projections

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39
Q

Vincristine
- mechanism
- side effect

A

Inhibits mitosis
S/E: peripheral neuropathy

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40
Q

Cyclophosphamide
- mechanism
- side effect

A

Promotes cross linking of DNA
S/E: haemorrhagic cystitis

41
Q

Doxorubicin
- mechanism
- side effect

A

Stabilises DNA topoisomerase which inhibits DNA/RNA synthesis
S/E: cardiomyopathy

42
Q

Bleomycin
- mechanism
- side effect

A

Degrades DNA
S/E: lung fibrosis - lower zones

43
Q

Cisplatin
- side effects (4)

A

Nephrotoxic
Ototoxic
Peripheral neuropathy
Hypomagnesaemia

43
Q

Causes of intravascular haemolysis (5)

A

Acute haemolytic transfusion reaction
G6PD deficiency
Cold AIHA
Paroxysmal nocturnal haemoglobinuria
Red cell fragmentation

44
Q

Extravascular causes of haemolysis (4)

A

Sickle cell disease
Thalassaemia
Haemolytic disease of the newborn
Warm AIHA

44
Q

What is the cause of a positive Schumm test?

A

Intravascular haemolysis

45
Q

Causes of cold AIHA (3)

A

Mycoplasma
EBV
Lymphoma

46
Q

Causes of warm AIHA (4)

A

Penicillin
Cephalosporins
Lymphoma
SLE

47
Q

Ch responsible for alpha chain in Hb

A

Ch 16

48
Q

Genetic issue in sickle cell disease

A

Amino acid substitution
Glutamate substitutes for valine at codon 6

49
Q

Ch responsible for beta chain Hb

A

Ch 11

50
Q

Lead poisoning
- microscope finding
- defect

A

Basophilic stippling
Defect in ferrochelatase ALA dehydratase

51
Q

Defect in sideroblastic anaemia
- finding

A

Delta aminolevulinate synthase 2
Sideroblasts

52
Q

What is the role of G6PD?

A

Promotes production of NADPH which promotes generation of gluthianone which protects RBC from oxidative damage

53
Q

Pathophysiology of paroxysmal nocturnal haemoglobinuria

A

Pig A mutation
Reduced GPI
Reduced DAF - results in unregulated complement damage and intravascular AIHA

54
Q

Investigation of paroxysmal nocturnal haemoglobinuria

A

Flow cytometry - see a lack of CD59/CD55

55
Q

What is the indirect coomb’s test used for?

A

Detects free and unbound antibodies against specific antigens

56
Q

Worst prognosis of Hodgkin’s lymphoma

A

Lymphocyte depleted

57
Q

What is the Schumm test?

A

When haptoglobin becomes saturated the Hb binds to albumin = methemalbumin

58
Q

What is the most common subtype of Hodgkin’s Lymphoma?

A

Nodular sclerosing

59
Q

Genetic mutation associated with Burkitt’s lymphoma

A

c-myc
8:14 translocation

60
Q

What is a stage 3 Hodgkin’s lymphoma?

A

= positive lymph nodes either side of the diaphragm

61
Q

What is JAK2?

A

It is a non-receptor tyrosine kinase which stimulates growth

61
Q

Percentage with myelodysplasia which progress to AML?

A

30%

62
Q

Why do you get splenomegaly in myelofibrosis?
- investigation finding

A

Bone marrow fibrosis means there is extramedullary haematopoiesis
Tear drop poikilocytes

63
Q

Primary polycythaemia =

A

= polycythaemia rubra vera

64
Q

Investigation findings in PRV (4)

A

Low ESR
High leukocyte ALP
High Hb
Low epo

65
Q

Management of essential thrombocytosis

A

Hydroxyurea

66
Q

Features suggestive of poor prognosis in AML (2)

A

<60 years
Deletions at Ch 5 or 7

67
Q

Investigation findings in AML (3)

A

Auer rods
>30% blasts in BM
15:17 in APML

68
Q

Induction chemotherapy in AML

A

Cytarabine + anthracycline
(then consolidation)

69
Q

Management of CML (2)

A

Imatinib
Hydroxyurea

70
Q

Investigation findings in CML (3)

A

Ch 9: 22 - philadelphia chromosome
= BCR-ABL proto-oncogene
Low leukocyte ALP

71
Q

Poor prognostic markers in CLL (2)
Association with CLL

A

TP53
CD38
Warm AIHA

71
Q

Richter’s transformation ?

A

Transformation of CLL to high grade NHL

72
Q

Management of CLL
- chemotherapy regimen

A

RT usually
Chemo = FCR
Fludarabine, Cyclophosphamide, Rituximab

72
Q

Good prognosis in CLL

A

13q14 deletion

73
Q

Poor prognostic markers in ALL (2)

A

4:11 translocation
Philadelphia chromosome

73
Q

Features of hyposplenism on blood film (3)

A

Howell Jolly bodies
Target cells
Pappenheimer bodies

74
Q

Features of IDA on blood film (2)

A

Target cells
Pencil poikilocytes

75
Q

Feature of megaloblastic anaemia on blood film

A

Hypersegmented neutrophils

76
Q

What is hairy cell leukaemia?

A

A subset of CLL

77
Q

Association of AL amyloidosis

A

Malignancy - MGUS, waldenstrom’s, myeloma

78
Q

Association of AA amyloid (3)

A

Chronic infection
SLE
TB

79
Q
A
79
Q

What is Waldenstrom’s macroglobulinaemia associated with?

A

Cryoglobulinaemia I (monoclonal proliferation)

80
Q

mGUS vs myeloma investigation finding

A

mGUS = normal B2 microglobulin

81
Q

Causes of acquired Haemophilia A (6)
- mechanism

A

Psoriasis
Malignancy
Pemphigus
IBD
Phenytoin
RA
= IgG antibodies with factor VIII

81
Q

Mechanism of action of TXA

A

Inhibits plasminogen conversion to plasmin

82
Q

Investigation findings in vWF (2)

A

Slightly raised APTT
Reduced factor VII

83
Q

Primary haemostasis
- 3 steps

A

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
Q

Problem in methahaemaglobinaemia

A

Fe2+ oxidised to Fe3+
= reduced capacity for O2 carrying, high affinity for oxygen, poor release of O2 at tissues

85
Q

Investigation finding in methahaemoglobinaemia
Management

A

ABG - normal PaO2 but low SaO2
Methylene blue

86
Q

What is the mechanism in ITP?

A

IgG antibodies against GP2b3a

87
Q

What is Evan’s syndrome?

A

ITP + AIHA

88
Q

Features of systemic mastocytosis (3)

A

Urticaria
Flushing
Darrier’s sign = wheals appearing on rubbing the skin

88
Q

Management of HIT?

A

Stop heparin
Do not give warfarin - can trigger necrosis skin reaction

89
Q

Genetic change in haemochromatosis

A

HFE gene on chromosome 6

90
Q

What can you reverse in haemochromatosis with treatment?

A

Skin pigmentation
DCM

91
Q

What do you need to do prior to elective splenectomy?

A

Vaccinations 2/52 before
Pneumococcus
Haemophilus influenza
Men A/C
Influenza

92
Q

Investigation findings in haemochromatosis (3)

A

Raised transferrin
Raised ferritin
Chondrocalcinosis

1MRCP Part 1 (20 decks)

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