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haem Flashcards

1
Q

management of post-thrombotic syndrome (swelling, varicose veins, pruritis, heavy calves)

A

compression stockings
keep leg elevated

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2
Q

the most common inherited clotting bleeding disorder

A

Von willebrand’s disease

  • usually prolonged bleeding after dental extraction
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3
Q

what is the mode of inheritance of von willebrand’s disease

A

autosomal dominant

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4
Q

there are 3 types of von willebrand disease - what differs between them

A

Type 1: partial vWF reduction (80%)
Type 2: abnormal vWF
Type 3: complete lack of vWF

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5
Q

what carrier molecule does von willebrand factor help

A

factor 8

it also promotes platelet adhesion

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6
Q

Prolonged PT
Prolonged APTT
Reduced factor 8
Defective platelet aggregation with ristocetin

Nosebleeds
Heavy periods

What is the diagnosis

A

von willebrand’s disease

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7
Q

Management of von willebrand’s disease bleeding

A

Tranexamic acid - for mild bleeding
Desmopressin
Factor 8 concentrate

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8
Q

Cancer patients with VTE treatment and length

A

6 months of DOAC

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9
Q

Patients taking bone marrow suppression drugs (especially methotrexate) should avoid which eye drops

A

CHLORAMPHENICOL

can cause aplastic anaemia

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10
Q

In cancer patients who are neutropenic, what type of exam should not be done

A

PR EXAM
avoid translocation of flora through rectal mucosa

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11
Q

what is neutropenic sepsis defined as

A

Neut <0.5 * 10^9

And one of:
temp >38 or
septic

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12
Q

What bacteria are the most common cause of neutropenic sepsis

A

Staphylococcus epidermidis

(Gram +ve coagulase negative)

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13
Q

Management of neutropenic sepsis

A

Tazocin
If unwell after 48 hours, add meropenem +/- vancomycin
If no response after 4-6 days, investigate for fungal infections e.g. CT-HR
Consider G-CSF

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14
Q

CRAB myeloma

A

Calcium high
Renal failure
Anaemia
Bony pain and bleeding

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15
Q

in people aged 60 and over with persistent bone pain, particularly back pain, or unexplained fracture, to assess for myeloma what tests should be done

A

FBC
Calcium
Plasma viscosity
ESR

then if these are positive, do bence-jones protein and protein electrophoresis

+ bone marrow aspiration
+ imaging for bone lesions (MRI whole body)

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16
Q

sickle cell patients need to pneumococcal polysaccharide vaccine how often

A

every 5 years

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17
Q

long term management of sickle cell anaemia

A

hydroxyurea - to increase HbF
pneumococcal vaccine every 5 years

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18
Q

tamoxifen therapy effect on the risk of VTE

A

increases risk of VTE

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19
Q

COCP - which generations have high risk of VTE

A

3rd gen > 2nd gen

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20
Q

which two hormone cancer medications increase risk of VTE

A

raloxifene
tamoxifen

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21
Q

DVT investigation: if the scan is negative, but the D-dimer is positive
what should be done

A

stop anticoagulation
repeat USS scan in 1 week

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22
Q

tamoxifen increases the risk of which cancer

A

endometrial cancer

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23
Q

which haemophilia accounts for 90% of cases of haemophilia

A

haemophilia A

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24
Q

what is the mode of inheritance of haemophilia

A

x-linked recessive

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25
Q

haemophilia A is a deficiency of…

A

factor 8

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26
Q

haemophilia B (Christmas disease) is a deficiency of..

A

factor 9

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27
Q

blood tests for haemophilia show what results for:
APTT
Bleeding time
thrombin time
prothrombin time

A

Prolonged ATT
Normal bleeding time, thrombin time, PT

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28
Q

Which antipsychotic has increased risk of VTE

A

olanzapine

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29
Q

If investigating a suspected DVT, and either the D-dimer or scan cannot be done within 4 hours, what treatment is given

A

Start a DOAC

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30
Q

If a 2-level DVT Wells score is ≥ 2 points then what should be done next

A

ultrasound scan WITHIN 4 hours

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31
Q

If a 2-level DVT Wells score is 1 point or less then what should be done next

A

D-dimer test

If positive, then do USS within 4 hours

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32
Q

Sickle cell patients with low Hb, low reticulocytes is usually due to what infection

A

PARVOVIRUS infection

This tends to cause aplastic crisis due to bone marrow suppression

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33
Q

Sickle cell anaemia
Parvovirus infection
Low Hb
Low reticulocytes

What type of crisis is this?

A

Aplastic crisis

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34
Q

4 types of sickle cell crisis

A
  1. Thrombotic i.e. vasoocclusive /painful
  2. Acute chest
  3. Anaemic
    - aplastic
    - sequestration
  4. Infection
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35
Q

Thrombotic crisis in sickle cell anaemia (aka painful or vasocclusive crisis) is triggered by what 3 things

A
  1. dehydration
  2. infection
  3. low O2 e.g. high altitude
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36
Q

Acute chest syndrome is a type of sickle cell crisis. Vasoocclusion happens where?

A

Within the pulmonary microvasculature
Leads to infarction in lung tissue

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37
Q

Shortness of breath
Chest pain
Pulmonary infiltrates on CXR
Low pO2

What type of sickle cell crisis is this?

A

Acute chest syndrome

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38
Q

What is the most common cause of death after childhood in sickle cell disease patients

A

Acute chest syndrome

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39
Q

Increased reticulocyte count
Worsening anaemia

What type of sickle cell crisis is this?

A

Sequestration crises

Sickling within organs e.g. spleen or lung worsens the anaemia

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40
Q

What is the difference in reticulocytes between sickle cell anaemia - anaemic crises
(a) aplastic
(b) sequestration

A

(a) aplastic - low reticulocytes (bone marrow suppression)
(b) sequestration - high reticulocytes

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41
Q

Immune thrombocytopenia (ITP) is an immune-mediated reduction in platelet count.
Antibodies are directed against what

A

Abs against Glycoprotein IIb/IIIa or Ib-V-IX complex

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42
Q

Children with ITP usually have acute thrombocytopenia (low platelets) that follows what

A

infection or vaccination

(adults tend to have a more chronic condition)

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43
Q

What does FBC show in immune thrombocytopenia (ITP)

A

Isolated low platelets

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44
Q

First line management of ITP

A

Oral prednisolone

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45
Q

What is Evan’s syndrome

A

ITP (low platelets) in association with autoimmune haemolytic anaemia

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46
Q

Management of ITP - three options

A
  1. Oral pred - 1st line
  2. Pooled normal human immunoglobin (IVIg) - acts faster than steroids so if active bleeding or urgent procedure needed
  3. Splenectomy - less common
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47
Q

3 causes of high APTT

A
  1. Heparin therapy
  2. Haemophilia (A = low factor 8, B low factor 9)
  3. Antiphospholipid syndrome
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48
Q

Chronic lymphocytic leukaemia (CLL) is caused by monoclonal proliferation of well-differentiated lymphocytes which are usually what cell

A

B-cells (99%)

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49
Q

What is the most common form of leukaemia in adults

A

chronic lymphocytic leukaemia (CLL)

50
Q

FBC shows what in chronic lymphocytic leukaemia (CLL)

A

Lymphocytosis (high lymphocytes)
Anaemia
Thrombocytopenia

51
Q

Blood film shows smudge/smear cells - what is likely diagnosis

A

Chronic lymphocytic leukaemia (CLL)

52
Q

What is the key investigation for chronic lymphocytic leukaemia - and what does it look for

A

Immunophenotyping

Uses Abs specific for CD5, CD19, CD20, CD23

53
Q

Antiphospholipid syndrome - in pregnancy what are the 6 issues that can occur

A
  1. recurrent miscarriage
  2. IUGR
  3. pre-eclampsia
  4. placental abruption
  5. pre-term delivery
  6. VTE
54
Q

management of antiphospholipid syndrome in pregnancy

A
  1. Low dose aspirin once pregnancy is confirmed
  2. LMWH once foetal heart seen on USS - discontinued at 34 gestation
55
Q

When is LMWH started and stopped in pregnancy with antiphospholipid syndrome

A

Starts: when foetal heart on USS
Stops: 34 weeks gestation

56
Q

G6PD deficiency is triggered by which types of drugs

A

Sulph- drugs: e.g. sulphonamides
Sulphasalazine
Sulfonylureas - gliclazide

Ciprofloxacin

Anti-malarials - primaquine

AND BROAD FAVA BEANS!

57
Q

What is the inheritance of Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

X-linked recessive

58
Q

What is the pathophysiology of G6PD deficiency

A

Low G6PD

Reduced NADPH production

NADPH helps to convert oxidized glutathine back to reduced form (which would protect RBCs from oxidative damage)

Increases RBC oxidative stress

59
Q

What type of anaemia occurs with G6PD deficiency

A

Intravascular haemolysis

Haemolytic anaemia

Neonatal jaundice occurs

60
Q

What is seen on blood films with G6PD deficiency

A

Heinz bodies
Bite and blister cells

61
Q

How do you diagnosis G6PD deficiency

A

G6PD enzyme assay

Check levels around 3 months after acute episode of haemolysis

62
Q

What is seen on blood film for:
(a) G6PD deficiency
(b) hereditary spherocytosis

A

(a) Heinz bodies (bite and blister cells also may be seen)
(b) Spherocytes

63
Q

Pernicious anaemia have which antibodies

A

Intrinsic factor antibodies - used for diagnosis
Gastric parietal cell antibodies

64
Q

What is the mechanism of action in pernicious anaemia of:
(a) Intrinsic factor antibodies
(b) Gastric parietal cell antibodies

A

(a) Intrinsic factor Abs bind to intrinsic factor blocking the vitamin B12 binding site
(b) Gastric parietal cell Abs reduce IF production and reduce acid production, leads to atrophic gastritis.

65
Q

Blood film findings with pernicious anaemia

A

Megaloblastic macrocytic anaemia
Hypersegmented polymorphs

66
Q

NICE specifically state that we should not use QRISK2 for type 1 diabetics. Instead, the four following criteria are used:

A

Older than 40 years, or
Has had diabetes for >10 years or
Has established nephropathy or
Has other CVD risk factors

n.b. start 20mg atorvastatin for these EVEN if their lipid screen is normal!

67
Q

Steven Johnson’s syndrome - Nikolsky sign shows …

A

In erythematous areas, blisters and erosions appear when the skin is rubbed gently

68
Q

Lymphadenopathy occurs in 75% of Hodgkin’s lymphoma. Where are the glands most commonly?

A

Neck
i.e. cervical/ supraclavicular

> axillary
inguinal

69
Q

Painless
Non-tender
Asymmetrical

Hodgkin’s or non-Hodgkin’s lymphoma?

A

Hodgkin’s lymphoma

70
Q

Pel-Ebstein fever relates to which condition

A

Hodgkin’s lymphoma

71
Q

What blood results are seen in Hodgkin’s lymphoma

A

Normocytic anaemia
High eosinophils
High LDH

72
Q

Lymph node biopsy for Hodgkin’s lymphoma shows which type of cells

A

Reed-Sternberg cells

these are diagnostic

73
Q

If you are suspicious of leukaemia in ages 0-24 years, e.g. pallor, fatigue, infections, what is an important test to do and timeframe?

A

FBC within 48 hours

74
Q

patients with inherited haemolytic anaemias e.g. hereditary spherocytosis and G6PD usually have jaundice alongside what other abdo presentation?

A

gallstones!

75
Q

patients with hereditary spherocytosis can be diagnosed without any special tests on the basis of what criteria..

A
  • Family history
  • Clinical features
  • Lab results e.g. Spherocytes,
    High MCHC,
    High reticulocytes
76
Q

to diagnose hereditary spherocytosis when the diagnosis is unsure, what two tests can be done

A

EMA binding test
cryohaemolysis test

77
Q

for ATYPICAL presentations of hereditary spherocytosis, what test can be done

A

electrophoresis analysis of erythrocyte membranes

78
Q

management of hereditary spherocytosis acute crises

A

supportive treatment
transfusion if neccessary

79
Q

management of hereditary spherocytosis acute crises is supportive with transfusion if needed.
what is used for longer term treatment?

A

folate replacement
splenectomy

80
Q

which lymphoma has more pain when drinking alcohol

A

hodgkin’s lymphoma

81
Q

what are 4 systemic B symptoms of lymphoma

A

Weight loss
Pruritis
Night sweats
Fever (Pel-ebstein)

82
Q

After correcting iron deficiency anaemia, iron replacement should be continued for how long, and when should it next be checked?

A

3 months then stopped

Then monitor FBC every 3 months for one year

83
Q

What is the profile of the following in iron def anaemia:
Serum iron
Ferritin
TIBC/transferrin
Trans sats

A

Serum iron - low
Ferritin - low
TIBC/transferrin - high
Trans sats - low

84
Q

anisopoikilocytosis (red blood cells of different sizes and shapes)
target cells
‘pencil’ poikilocytes

what is the condition

A

iron deficiency anaemia

85
Q

Patients with iron def anaemia - it is important to refer for 2ww endoscopy to rule out GI cancer. Who is referred?

A

Post-menopausal women with Hb <10
Men Hb <11

Age over 60 with IDA

86
Q

Management of iron def anaemia

A

Oral ferrous sulfate
Take for full 3 months even if it has been corrected
Then recheck - if normal then stop iron
Monitor FBC every 3 months for one year thereafter

87
Q

Acute myeloid leukaemia FBC profile

A

Low Hb - anaemia
Low platelets
Low neutrophils

(blood fim - bilobed large mononuclear cells)

88
Q

Poor prognostic features of acute myeloid leukaemia

A

> 60 years

> 20% blasts after first course of chemo

Genetics: Chr 5 or Chr 7 deletions

89
Q

Acute promyelocytic leukaemia M3 is associated with which Chr translocation

A

t(15;17)

90
Q

PML and RAR-alpha genes is seen with which cancer

A

Acute promyelocytic leukaemia M3 (type of AML)

91
Q

Auer roads (seen with myeloperoxidase stain) is associated with which cancer

A

Acute promyelocytic leukaemia M3 (Type of AML)

92
Q

t(15;17) is seen with what condition

A

Acute promyelocytic leukaemia M3 (Type of AML)

93
Q

There are 8 types of acute myeloid leukaemia - what classification is it

A

French-American-British (FAB)

M0 - M7

94
Q

vitamin B12 is absorbed after binding to …

A

intrinsic factor

secreted from parietal cells in the stomach
and is absorbed in terminal ileum

95
Q

what is the most common cause of vit B12 def

A

pernicious anaemia

96
Q

vit B12 deficiency can cause neurological symptoms - what is usually affected first

A

dorsal column
i.e. joint position, vibration

prior to distal paraesthesia

97
Q

management of vitamin B12 deficiency if no neurological involvement

A

1mg IM hydroxocobalamin

3 times each week for 2 weeks

Then once every 3 months

98
Q

Drug induced pancytopenia from bone marrow suppression can occur from some medications e.g. carbimazole, sulphonylureas, trimethoprim, chloramphenicol, carbamazepam etc

What symptoms may the patient develop

A

Sore throat
Nose bleeds/menorrhagia
Pancytopenia

99
Q

Primary causes of polycythaemia (high Hb)

A

polycythaemia rubra vera

100
Q

Secondary causes ofpolycythaemia (high Hb)

A

COPD
High altitude
Obstructive sleep apnoea
Excess EPO: haemangioma, hypernephroma, hepatoma, fibroids

101
Q

To work out if it is TRUE polycythaemia versus relative (i.e. dehydration), what studies are used

A

Total red cell mass studies

In true polycythaemia, RBC mass increases:
Males >35
Women >32

102
Q

polycythaemia rubra vera is primary polycythaemia. what gene has the mutation

A

JAK2 gene mutation

103
Q

How to distinguish between primary and secondary true polycythaemia causes

A

Primary polycythaemia - reduced EPO
Secondary polycythaemia - increased EPO

104
Q

What is the most common cause of thrombophilia (clotting) disorder

A

Factor V Leiden

i.e. activated protein C resistance

105
Q

Factor V Leiden is a disorder that leads to what clotting issue

A

Activates protein C resistance

106
Q

5 inherited clotting disorders

A
  1. Factor V Leiden
  2. Prothrombin gene mutation
  3. Antithrombin 3 deficiency
  4. Protein C deficiency
  5. Protein S deficiency
107
Q

Acquired clotting disorder

A

Antiphospholipid syndrome

108
Q

which antibiotic leads to jaundice and haemolysis in G6PD

A

CIPROFLOXACIN

109
Q

3 key changes in NICE 2020 VTE guidelines

A
  1. Use of DOACs as first line
  2. DOACs should be used for VTE in active cancer patients
  3. Routine cancer screening is NOT recommended after VTE diagnosis
110
Q

Patient with VTE with renal impairment that is severe e.g. eGFR <15, what should be given to treat it?

A

LMWH
Unfractionated heparin
Or LMWH with VKA

111
Q

Unprovoked VTE treatment duration

A

6 months

112
Q

Provoked VTE treatment duration

A

3 months

113
Q

hereditary spherocytosis inheritance mode

A

autosomal dominant

114
Q

what antiepileptic drug interacts with DOACs

A

carbamazepine

115
Q

In myeloma, what is seen on peripheral blood film?

A

Rouleaux formation

116
Q

Protein electrophoresis shows raised concentrations of what in myeloma

A

Monoclonal IgA/IgG proteins

117
Q

X-rays: ‘rain-drop skull’
what is this seen in?

A

Myeloma

118
Q

X-ray - pepperpot skull
what is this seen in?

A

Primary hyperparathyroidism

119
Q

What 8 symptoms in a person aged from 0-24 years prompt an urgent FBC within 48 hours to look for leukaemia?

A
  1. Pallor
  2. Persistent fatigue
  3. Unexplained fever
  4. Persistent infections
  5. Lymphadenopathy
  6. Bone pain
  7. Unexplained bruising
  8. Unexplained bleeding
120
Q

What does FBC show in IDA

A

Hypochromic microcytic anaemia

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