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Flashcards in More CP Deck (92):
1

What is haematocrit?

Vol % rbcs in blood

2

Is haematocrit higher or lower in children?

Higher

3

Zeta + epsilon = what sort of Hb?

Hb Gower-1

4

Zeta + gamma =?

Hb Portland

5

Alpha + epsilon

Hb gower-2

6

Hb gower 1 is ?

Zeta + epsilon

7

Hb Portland is ?

Zeta + gamma

8

Hb gower 2 is ?

Alpha + epsilon

9

When are Hbs gower1, portland and gower 2 present?

4-14 weeks gestation

10

When does HbF become present?

more than 14 weeks gestation

11

Difference in WBCs in children?

More lymphocytes

12

Which Ig crosses the placenta?

IgG

13

Which Igs in breast milk?

All

14

When do babies start producing their own antibodies?

2-3 months

15

When can babies have their own satisfactory immune responses?

6 months

16

When do platelets reach adult numbers?

18 weeks gestation

17

What are 2 features of gestational platelets?

Larger initially but reduce to adult size by birth
Hyperresponsive to vWF

18

Which clotting factors are normal at birth? What are the rest of them like?

Fibrinogen, FV, VIII, XIII
Rest are reduced

19

When do haemostatic parameters reach adult values?

6 months

20

What is vitamin k dependent other than factors 10, 7, 9, 2?

Proteins S and C

21

What does routine neonatal vit k injections prevent?

Haemorrhagic disease of the newborn

22

What are 5 features of neonatal haemostasis?

Pro-coagulant proteins reduced (e.g. all the other factors)
Reduced conc. coagulation inhibitors
unique forms fibrinogen, plasminogen
Raised D dimers and vWF
Platelet aggregation differs

23

What is acute leukaemia?

Accumulation of early myeloid or lymphoid precursors in BM, blood and other tissues

24

What are the two main subgroups of acute leukaemia

Acute myeloid leukaemia and acute lymphoblastic leukaemia

25

How does acute leukaemia present?

Anaemia
Infection
Easy bruising or haemorrhage
Possibly organ infiltration by the leukaemia cells

26

FAB classification of leukaemia is ?

Morphological

27

WHO classification of leukaemia is ?

Risk adapted- takes into account prognosis

28

6 poor prognostic factors in ALL?

Age
High wcc
Male
Cytogenetic abnormalities (some)
Poor response to Rx
T cell -ALL and null-ALL

29

2 tests for diagnosis?

MABs
Fluorescence activated cell sorting

30

Treatment for acute leukaemia that is shared with ALL and AML

Induction therapy to obtain remission
Consolidation cheomtherapy
Possible BM transplant

31

Additional Rx in ALL

Prophylaxis of meningeal leukaemia with intrathecal methotrexate and cranial irradiation

32

Siblings have a ?% change of being a bone marrow donor match?

25%

33

What is neutropenic sepsis?

All pts with acute leukaemia receiving intensive chemotherapy will become neutropenic for 10-21 days.
Overwhelming gram negative or positive infection= sepsis.

34

What is neutropenic fever?

Pyrexia in the presence of neutrophil count of less than 1.0/10^9/L

35

What is the management of neutropenic sepsis?

Immediate broad spectrum IV Abx (often Tazocin and gentamicin) given empirically- within 30 mins of temperature

36

Prevention of neutropenic sepsis?

Isolation
Prophylactic Abx
Granulocyte colony stimulating factors
Strict hand hygeine

37

Which element of virchow's triad contributes to arterial thrombosis?

Endothelial damage

38

Which elements of virchow's triad contributes to venous thrombosis?

Venous stasis and hypercoagulable state

39

What are arterial thrombi made of?

Platelets

40

What are venous thrombi made of?

Fibrin

41

Definition of a hospital acquired VTE?

Within 90 days discharge

42

Having considered the risks of bleeding and VTE, if there is a risk of bleeding how can you still help prevent a VTE?

Mechanical methods eg stockings

43

When must you reassess risks of bleeding and VTE?

Within 24hrs admission and whenever the clinical situation changes

44

True of false antiplatelets are adequate prophylaxis against VTE alone?

False, also mobilisation and hydration

45

4 pharmacological methods of prevention

1. low molecular weight heparin (antithrombin and mostly anti-Xa)
2. Fondaparinux
3. Rivaroxaban/apixaban (direct factor Xa inhibitor)
4. Dabigatran (direct thrombin inhibitor)

46

What is the exclusion test for a VTE?

Use Wells score and D-dimer in an algorithm

47

What are D-dimers?

Breakdown product of fibrin clots

48

Scans for:
VTE
PE

VTE- US
PE- CT, VQ- compare inhaled and injected isotopes. Mismatch is diagnostic of a PE

49

Advantages of Rivaroxaban/apixaban/dabigatran over heparin?

Dose uniform rather than done by weight so monitoring not needed
Rapid onset of action
Oral route

50

Management for a first episode of DVT or PE

Treat for 3-6 months if there is a temporary risk factor such as pregnancy

51

What is warfarin target INR?

2.5

52

Management for recurrent episodes of cardiothoracic emboli?

Long term anticoagulation

53

Management of a proximal DVT or PE that has occured in the absence of a reversible risk factor?

Consider long term anticoagulation

54

Management if they have recurrent VTEs even on therapeutic anticoagulants?

Increase target INR to 3.5 for warfarin

55

What is a thrombophilia (2 definitions)

1. familial/acquired predisposition to thrombosis
2. patients who develop VTE spontaneously, extra bad or recurrently at an early age

56

What is the action of protein C and what is it activated by?

Thrombin-thrombomodulin complex activates it (thrombomodulin is on the vessel wall), protein C-protein S complex breaks down factor Va and VIIIa resulting in coagulation.

57

In factor V leiden, factor V is resistant to what?

Protein C

58

When is factor V leiden asymptomatic?

Heterozygous

59

Are screening tests valuable in factor V leiden?

Debatable as often wouldn't change management. Doesn't increase risk of recurrent thrombosis.

60

What is prothrombin 20210A?

Increased prothrombin levels, 3 fold increase in VTE

61

What is the dx criteria for antiphospholipid syndrome?

Presence of antiphospholipid antibodies on at least 2 occasions 8-12 weeks apart in assoc with VT, AT or recurrent foetal loss (more than 2)

62

What might antiphospholipid syndrome be secondary to?

SLE, conn tissue disorders etc.

63

When should you screen for thrombophilia?

In younger patients, positive Fhx and if management will change.

64

Folate and B12 deficiency cause what type of anaemia?

Megaloblastic macrocytic

65

Main cause and other 4 causes of folate deficiency?

Dietary main cause
Alcohol
Malabsorption
Increased usage
Drugs

66

What is iron transported in?

Transferritin

67

What is iron stored in?

Ferritin/haemosiderin

68

2 causes of microcytic anaemia

Fe deficiency
Hb disorders

69

3 causes of iron deficiency

Blood loss
Increased demand (pregnancy,growth)
Redued intake (diet, malabsorption)

70

Rx for iron deficiency

Oral iron (although constipation and abdo pain)
IM iron (although painful and skin colour change)
IV iron probably best

71

How do B12 and folate deficiencies cause anaemia?

B12 required for cell folate generation, folate required for nitrogenous base synthesis which make up DNA

72

What foods is B12 from?

Animal sources

73

Describe the absorption of B12

Gastric parietal cells absorb
Bound to intrinsic factor
Receptors in terminal ileum

74

How long are B12 stores sufficient for?

Years

75

3 causes of B12 deficiency

Nutritional (vegan)
Gastric problems (pernicious anaemia, gastrectomy)
Small intestine problems (resection, crohns, stagnant loops, jejunal diverticulitis, tropical sprue, fish tapeworm)

76

Severe B12/folate deficiency can result in...?

pancytopenia

77

What neurological problem can B12 deficiency result in?

Sub acute degeneration of the spinal cord

78

Rx B12+/folate deficiency?

Give B12 and folate until B12 deficiency is excluded as folic acid in isolation can make B12 def worse.

79

7 cases haemolytic anaemia

Haemoglobinopathy
Enzyme defects (G6PD)
Hereditary spherocytosis/elliptocytosis
Antibodies to RBC
Drubs/toxins
Heart valves
Vascular

80

What is the name of haemolytic anaemia in its own right?

autoimmune haemolytic anaemia (AIHA)

81

Rx AIHA

steroids and immune suppression

82

What sort of aneamia is anaemia of chronic disease?

Normocytic

83

What is the MCV in anaemia of chronic disease?

Normal

84

3 reasons anaemia of chronic disease happens?

Abnormal Fe metabolism
Poor EPO response
Poor BM response

85

11 causes of thrombocytopenia

drugs alcohol toxins
infection (sepsis, EBV, HIV)
Other autoimmune
DIC
Liver disease
Hypersplenism
Congenital
Pregnancy
Haematological/marrow diseases
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura

86

Immune thrombocytopenic purpura cause in children

Post viral- self limiting

87

Rx immune throbocytopenic purpura

Steroids
Thrombomimetics
IV Igs (blocks spleen)
Splenectomy

88

What is the role of the spleen in terms of platelets?

Removes them

89

How does Immune thrombocytopenic purpura present?

Bruising, petechiae, bleeding
Splenomegaly

90

Cause of thrombotic thrombocytopenic purpura

Immune (ADAMTS-13, vWF)

91

Presentation of thrombotic thrombocytopenic purpura

Thrombocytopenia +
Fever, neurological, haemolysis

92

Rx thrombotic thrombocytopenic purpura

Plasma exchange
Steroids