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Fifth Year Medicine > Haematology > Flashcards

Haematology Flashcards

1
Q

What is myelodysplasia and what is the risk?

A

Ineffective blood cell production due to bone marrow failure, from fibrosis or lymphocytic infiltration of the marrow.

30% transform to become Acute Myeloid Leukaemia

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2
Q

Elderly lady at her GP is found to have a high white blood cell count, not acutely unwell, what is the likely cause?

A

Chronic lymphocytic leukaemia

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3
Q

Auer rods are characteristic of which blood cancer?

A

Auer rods? My rods

CML

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4
Q

Carbot rings occur in the blood film of people who have suffered poisoning from what?

A

Lead poisoning, Carbot rings may be microtubules gone awry

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5
Q

Which virus triggers an aplastic crisis in sickle cell patients, and what happens when this occurs?

A

Parvovirus B19

Reduction in RBCs

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6
Q

Drug causes of spherocytosis (Coombs +ve)?

MNOPQ

A 
Methyldopa
NSAIDs
Oh IFN!
Penicillin
Quinine
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7
Q

Low Hb, raised bilirubin
Blood film shows: Spherocytosis, Coombs +ve
Not worsened by cold
What could be the cause and Rx?

A

Warm haemolytic anaemia, spherocytes = spherical cells
Coombs = autoimmune, Warm = IgG

Idiopathic
CLL, Lymphoma
Autoimmune, SLE

High dose prednisolone

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8
Q

Infectious causes of Coombs +ve cold autoimmune haemolytic anaemia (what type of Ig mediates it?)

A

IgM- mycoplasma pneumoniae, EBV

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9
Q

A patient has been on holiday and over the last few days has had rigors, developed jaundice and reports black urine.
FBC shows progressive anaemia. Cause? Rx?

A 
Blackwater fever (malaria) due to massive intravascular haemolysis, possibly from autoimmune reaction.
Rx: antimalarials
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10
Q

Things someone with Glucose 6 Phosphate Dehydrogenase should avoid and disease inheritance?

A

X linked, G6PD is an enzyme protecting from oxidative stress

Aspirin, sulphonamides
Broad beans
Henna, dapsone (coeliac rash)

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11
Q

Cause of severe haemolytic uraemic syndrome? Possible complications?

A

E Coli 0157, produces Shiga toxin that stimulates cytokines promoting clot formation and inactivates ADAMTS13 protease (needed to cleave vWF to prevent multimers + clots)

Kidney failure, due to clots in small vessels + bloody diarrhoea

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12
Q

In someone with von willebrand’s disease, what FBC and clotting studies would be expected?

A

vWF binds factor 8 (intrinsic cascade) to carry it, promoting adhesion of platelets to damaged endothelium.

Reduction in levels leads to prolonged bleeding time
APTT may be prolonged (as less factor 8 carried)
Hb or MCV may be low if bleeding leads to iron deficiency

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13
Q

How do platelet disorders and coagulation disorders present differently in regards to symptoms?

A 
Platelet disorders (which includes vWF disease) = epistaxis and menorrhagia
Coagulation disorders are more severe = haemoarthrosis and muscle haematomas
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14
Q

68 year old patient has splenomegaly, lymphadenopathy and IgM paraprotein level of 40g/L. Normal calcium and creatinine.

Likely diagnosis?

A

Waldenstrom’s macroglobulinaemia
IgM paraprotein raise= less likely to be multiple myeloma (IgG, IgD would be more likely)
And other findings are not suggestive
Monoglonal gammopathy requires paraprotein of no more than 30g

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15
Q

Those with polycythaemia vera typically get intense itching when? What other conditions are associated?

A

When warm or humid (ie getting out the shower, due to histamine release by the RBCs)

Gout and DVTs

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16
Q

What findings indicate tumour lysis syndrome, how can you prevent it?

A

Hyperkalaemia (cell breakdown)
Hyperuricaemia (purine breakdown)
Hyperphosphataemia (nucleotide breakdown)

Allopurinol

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17
Q

Mutation causing polycythaemia rubra vera?

A

JAK2 (in 90%)

RBCs are more responsive to GFs like erythropoietin and do not apoptose if EPO is absent

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18
Q

Cause of polycythaemia?

A

Primary- JAK2 mutation (= rubra vera)

Secondary, hypoxia- altitude, COPD, cyanotic heart disease, smoking

Secondary, too high EPO- renal or hepatocellular carcinoma

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19
Q

Which infections are associated with non-hodgkins lymphomas?

A

EBV- Burkitts
HTLV- T cell
HCV, HHV8- Castleman’s
H Pylori- MALT

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20
Q

What are the diagnostic criteria of multiple myeloma?

Name 4 things

A
  1. Serum/urine electrophoresis- monoclonal band
  2. Marrow biopsy- more plasma cells
  3. End organ damage: hypercalcaemia, renal insufficiency, anaemia
  4. Bone lesions via xray or PET
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21
Q

What is the pathogenesis of primary amyloidosis?

A

Myeloma- proliferation of plasma cell clone
> Amyloidogenic monoclonal Ig
> Extracellular fibrillar light chain deposits
> Resistant to degradation
> Organ failure

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22
Q

What are the different types of amyloidosis?

A

AL amyloid (primary) due to Ig chains from myeloma

AA amyloid due to acute phase protein from chronic inflammation

Familial due to mutations in ‘prealbumin’

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23
Q

IHx for amyloidosis?

A

Biopsy and Congo Red stain: birefringent light makes it apple green

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24
Q

Which blood clotting test does haemophilia effect?

A

Prolonged aPTT

As haemophilia A (F 8) and haemophilia B (F 9) both affect the intrinsic pathway (factors 12, 11, 9 and 8)

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25
Q

Haemophilia A and haemophilia B have what kind of inheritance, and which clotting factor is affected?

A

Haemophilia A- factor 8
Haemophilia B- factor 9

Acquired haemophilia- antibodies against factor 8

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26
Q

Which clotting factors are vitamin K dependent?

A

2, 7, 9, 10

2 (prothrombin) + 10 = aPTT + PT
7 = PT
9 = aPTT

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27
Q

What agents are added to plasma samples to test PT, aPTT and thrombin time?

A

PT- thromboplastin
aPTT- Kaolin
Thrombin time- thrombin

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28
Q

How does Warfarin work?

A

Inhibits the reductase enzyme needed to regenerate vit K to make clotting factors 2, 7, 9 and 10

(And also some anti-coagulation factors, hence why warfarin has an initial pro-thrombotic effect and should be covered with heparin until day 5)

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29
Q

Target INR when someone has prosthetic heart valves?

A

3-4

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30
Q

Target INR with DVT or AF?

A

2-3

Can give aspirin if falls risk is high, but is less effective

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31
Q

Once a patient has had a DVT, how long should you continue anticoagulation?

A

If cause has gone away
6 weeks for below knee DVT
3 months for above knee DVT or PE

6 months if no cause found
Indefinitely if cause is enduring, ie thrombophilia

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32
Q

When should you stop Warfarin? (What PC or INR?)

When should you give vit K?

A

Stop Warfarin when INR is above 6

Give Vit K if INR above 8 + RFs for bleeding

Give vit K and prothrombin complex concentrate if major bleed

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33
Q

Which conditions are made worse by steroids?

DiTCH OP

A 
Diabetes
TB
Chickenpox
Hypertension
Osteoporosis
Peptic Ulcers
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34
Q

What medication works via an opposing mechanism to azathioprine?

A

Allopurinol inhibits xanthine oxidase

Azathioprine metabolised to mercaptopurine, broken down by xanthine oxidase (so leads to toxicity if given with allopurinol)

35
Q

11 year old in last few hours has noted petechiae and purpura coming out along legs and trunk.
FBC: low platelets only
What tests would be prudent?

A

Idiopathic thrombocytopenic purpura
IHx: film to ensure no other abnormalities

Rx: consider steroids or Ig
Life-threatening bleed = platelet transfusion

36
Q

Management of chronic ITP in children?

A

Splenectomy stops sequestration of antibody coated platelets in spleen
Rituximab and anti-D may reduce need for splenectomy

37
Q

What features present with purpura make ITP less likely?

A 
Abnormalities on blood film (aside from low platelets)
Mucosal bleeding
Lymphadenopathy
Hepatosplenomegaly
Pancytopenia
38
Q

Way to remember Rx for hODgkINs?

A

BleOmycin
Daunorubicin
Decarbazine
VINcristINe

39
Q

Which blood test indicates activity level of lupus?

A

Complement levels

40
Q

What blood test is important to do if investigating anaemia aside from FBC? And what does it indicate?

A

Reticulocyte count
If low in the context of anaemia = underproduction of RBCs
If high = haemorrhage, haemolysis etc

41
Q

How does the spleen indicate whether an anaemia is acute or chronic haemolysis?

A

Large spleen = chronic

42
Q

Why does G6PD deficiency cause a haemolysis?

A

RBCs need some housekeeping enzymes to survive for 3 months, if it lacks G6PD (X linked) then increased oxidative stress from drugs or infection or fava beans (broad beans which have a little fungus on them) will cause rapid death to RBCs

(IHx: Heinz bodies due to Hb precipitating in RBC )

43
Q

Why do Heinz bodies form in G6PD deficiency attack?

A

Without G6PD, the RBC can’t deal with oxidative stress and prevent the Hb becoming oxidised into mecaptohaemaglobin, which forms precipitating lumps visible as ‘golf ball’ patterned Heinz bodies

44
Q

What is the difference between HbA, HbA2 and HbF?

A 
HbA = a2b2
HbF = a2y2 (gamma)
HbA2 = a2d2 (delta)
45
Q

In thalassaemia, if there chains are not being used (due to a mutation in the matching a or b chains) how does the precipitation of these unpaired globin cause symptoms?

A

If precipitating in RBCs = haemolysis
If precipitating in bone marrow = ineffective erythropoetis

= anaemia = erythopoetin = bone marrow expansion, iron and skeleton deformities

46
Q

What do children with beta-thalassaemia major die from?

A

Iron overload

Require chelation

47
Q

Mother comes in for her antenatal results where they did FBC, which showed normal Hb but microcytic. What could the cause be?

A

Thalassaemia

Antenatal screening test includes Hb electrophoresis so would pick this up hopefully

48
Q

What is the pathology of damage caused by iron overload?

A

Iron exceeds the transferrin ability to bind it, so it travels free in the blood, is selectively uptaken by organs and promotes hydroxyl free radical production = damage
(Heart failure, endocrine failure)

49
Q

When chelating iron in iron overload (ie in blood transfusion Rx) what is the target ferritin?

A

Below 1000

The chelators have some bad side effects, neurotoxicity etc so can’t go mad with them

50
Q

Why does a RBC with sickle mutation sickle?

A

HbS polymerise into crystals in low O2 sats, causing characteristic shape.

51
Q

Why does hydroxyurea help prevent vaso-occlusive crises in children with sickle cell?

A

It increases levels of HbF

52
Q

What is the chromosome translocation in Burkitt’s lymphoma

A

8,14

Occurs in HIV + Africans

53
Q

Which type of myelocytic lymphoma is associated with bleeding problems, what therapy can be given?

A

Acute Promyelocytic Myeloid Leukaemia

Ie get DIC

54
Q

What chromosome translocation occurs in acute promyelocytic leukaemia?

A

15, 17

Responsive to all-transretinoic acid

55
Q

Multiple myeloma diagnoses require 1 major and 1 minor criteria, or 3 minor criteria.
What are the major criteria?

A

Major criteria:
Plasmacytoma- biopsy specimen (mass of abnormal plasma cells)
30% plasma cells on bone marrow biopsy
Serum or urine electrophoresis- elevated levels of Monoclonal protein

56
Q

For multiple myeloma diagnoses, 1 major + 1 minor criteria needs fulfilling or 3 minor. What are the minor criteria?

A

Minor criteria:
Bone marrow sample- 10% to 30% plasma cells
Plasma/urine electrophoresis- minor elevations in the level of M protein
Osteolytic lesions on imaging (xrays of chest, spine, pelvis + skull)
Low levels of other types of antibodies

57
Q

Which diseases are worsened by steroids?

A

Increase monitoring:
TB, chickenpox
Hypertension, diabetes
Osteoporosis

58
Q

Which medications reduce prednisolone concentration?

A

Anti-epileptics

Rifampicin (TB)

59
Q

What steps might reduce adrenal suppression if giving steroids?

A

Giving steroids alternate days
Giving steroids in the morning
Use lowest efficacious dose

60
Q

3 golden rules if on long term steroids:

A
  1. Carry a steroid card or bracelet
  2. Increase doses when sick
  3. Increase doses when stressed
61
Q

What dose of prednisolone warrants gradual withdrawal (due to adrenal suppression) rather than abrupt stopping?

Assuming the patient has been taking them > 3 weeks

A

7.5mg a day

62
Q

When would gradual weaning off steroids be needed if it’s a a short term treatment? (Under 3 weeks in length)

A

PMH: previous adrenal suppression
DHx: repeated courses of steroids
doses at night
>40mg dose

63
Q

What dose of steroids warrants gradual weaning even if given as a short term course? (Ie under 3 weeks)

A

> 40mg dose prednisolone

PMH: previous adrenal suppression
DHx: repeated courses of steroids
doses at night

64
Q

A patient is taking ciclosporin (calcineurin inhibitor) for transplant immunosupression. What is the main SE to worry about?

A

Dose-dependent nephrotoxicity

Other SEs: raised BP, oedema
Confusion, seizures
Gum hypertrophy, skin cancer

65
Q

Patient has had a transplant and is complaining of gum overgrowth (hypertrophy), what might the cause be?

A

Ciclosporin (calcineurin inhibitor)

66
Q

What blood test monitoring is needed for patients taking ciclosporin (calcineurin inhibitor used in transplant immunosuppression)?

A

U+Es and creatinine every 2 weeks for 3 months

(As nephrotoxicity is the main SE)

Also LFTs as hepatotoxicity can occur

67
Q

What change in creatinine warrants a dose reduction in a patient taking ciclosporin for Immunosuppression?

A

Increase of 30%, even if within the normal range

68
Q

What blood test is worth doing if a patient is taking cyclophosphamide?
(Given for R-CHOP in Non-Hodgkins, or for CLL)

A

Cyclophosphamide = alkylating agent

Causes marrow suppression - do FBC

69
Q

Patient is taking R-CHOP for Non Hodgkins lymphoma, suddenly starts peeing blood and has increased urinary frequency, what could be the cause?

A

Haemorrhagic cystitis: from cyclophosphamide (alkylating agent) of the R-CHOP

70
Q

Name the 4 commonest inherited causes of thrombophilia

A
  1. Deficiency in protein S or C (breaks down factor 5 and 7)
  2. Activated Protein C Resistance/ Factor 5 Leiden (factor 5 resists protein C breakdown)
  3. Prothrombin mutation (increases levels available to convert to thrombin)
  4. Antithrombin deficiency (inhibits thrombin)
71
Q

Which disease is associated with anti-phospholipid syndrome?

A

SLE

72
Q

When is testing for inherited causes of thrombophilia warranted?

A

In those with unprovoked DVT or PE if unsure whether to stop anticoagulation

May not be any great benefit to doing so, especially if patient will be on lifelong anticoagulation anyway

73
Q

Patient has a swollen red leg, had a resection of their bladder tumour a few days ago. What are the two next steps?

A

DVT might have occurred

Do a Wells score- 2 or more = likely
As cancer and recent surgery, they will have a high score

Do a proximal leg ultrasound if available within next 4 hours

74
Q

Patient is suspected of DVT, has a Wells score of 2. The hospital has no ultrasound technicians available for at least 8 hours. What test and Rx should be commenced?

A

A D-dimer and a parenteral anticoagulant

USS should be done within 24 hours

75
Q

Patient is suspected of a DVT, has a Wells score of 1. What IHx should be performed?

A

D dimer

If positive do USS within 4 hours or within 24 hours with an anticoagulant given

76
Q 
Patient is suspected of DVT
Wells score is 2
d dimer +ve
Leg USS -ve
What further management is needed?
A

Repeat leg ultrasound at 1 week incase of early but propagating DVT

77
Q

Patient has an unprovoked DVT and you want to rule out a underlying malignancy, what simple tests should you do?

A 
Urine dip (bladder)
FBC
LFTs
Ca (myeloma)
CXR (lung)
± CT abdo pelvis, mammogram
78
Q

A patient is deemed at risk of DVT due to increased immobility from her neck of femur fracture. Why might TED stockings be unsuitable?

A

Evidence of ischaemia

Absent posterior tibial or pedal pulses
Shiny skin or loss of hair

79
Q

3 causes of slow flow retinopathy- ‘feels like looking through a watery car windscreen’

A
  1. Hyperviscosity syndrome (high RBCs, WBCs, Igs-myeloma)
    May have confusion, abdo pain, lethargy also
  2. Carotid disease
  3. Takayasu’s disease (aorta arteritis, thrombosis, aneurysm)
80
Q

A patient has known multiple myeloma and reports reduced vision and a curtain falling over their sight. Aside from a stroke what might be the cause?

A

Hyperviscosity syndrome- high level of plasma componants

81
Q

Causes of massive splenomegaly:

A

My mate chrissy looks grouchy

Myelofibrosis
Malaria
CML
Leishmaniasis
Gaucher's (genetic enzyme defect causing lipid storage dysfunction)
82
Q

What 3 features differentiate the spleen from an enlarged kidney if there is a mass in the left upper quadrant?

A
  1. It moves down on inspiration (kidney is retro-peritoneal)
  2. You may feel the medial notch
  3. ‘You can’t get above it’- top margin disappears under the ribs
83
Q

What characteristic blood film is seen in someone post-splenectomy?

A

Normally the spleen removes them:

Howell-Jolly bodies (residual nuclear fragments)
Pappenheimer cells (iron granules)
Target cells

84
Q

What types of bacteria tend to be a problem in splenectomy patients?

A

Encapsulated ones:
Strep pneumo
Haemophilus influenzae
Neisseria meningitidis

Fifth Year Medicine (32 decks)

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