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haemotology and immunology > general > Flashcards

general Flashcards

1
Q

haemophilia A and B transmission

A

X-Linked

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2
Q

risk of inheriting haemophilia A

A

1/10,000

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3
Q

risk of inheriting haemophilia B

A

1/60,000

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4
Q

haemophilia complications

A

viral infections, anti Factor 8 antibodies, MI with DDAVP

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5
Q

epidemiology of von Willebrand disease

A

1/200

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6
Q

transmission of Von Willebrand disease

A

autosomal

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7
Q

type 1 von Willebrand disease refers too

A

quantitative deficiency

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8
Q

type 3 von Willebrand disease refers too

A

complete deficiency

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9
Q

3 examples of acquired bleeding disorders

A

liver failure, renal failure, warfarin

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10
Q

Ecchymosis refers too

A

bruise

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11
Q

ABO gene encodes for

A

glycosyltransferase

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12
Q

A and B genes code for

A

transferase enzymes

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13
Q

A antigen is

A

N-acetyl-galactosamine

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14
Q

B antigen is

A

galactose

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15
Q

out of A, B and O which is recessive and which is dominant?

A

A-B co dominant, O recessive

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16
Q

blood donors screened for

A

Hep B, C, E, HIV, syphilis

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17
Q

1 unit of RBC’s =

A

5g/L

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18
Q

RBC’s transfusion time

A

2-4 hours

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19
Q

shelf life of platelets

A

7 days

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20
Q

Fresh frozen plasma for a bleeding patient with factor 8 or 10 deficiency

A

no concentrate is available

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21
Q

Fresh frozen plasma for a bleeding patient on warfarin

A

no concentrate and Vit K

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22
Q

bleeding patient on DOAC use fresh frozen platelets?

A

no

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23
Q

a patient with DIC use fresh frozen platelets?

A

no

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24
Q

bleeding patient with DIC use fresh frozen platelets?

A

yes

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25
Q

patient with massive haemorrhage but no coagulation results use fresh frozen platelets?

A

yes

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26
Q

hypotensive patient with low albumin use fresh frozen platelets?

A

no

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27
Q

epitope spreading refers too

A

breakdown of self tolerance, the resulting inflammation releases further self peptides and thus more inflammation

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28
Q

if the AIRE gene is mutated what kind of tolerance can’t take place?

A

central

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29
Q

AIRE gene refers too

A

Autoimmune Regulator

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30
Q

peak onset for an autoimmune disease is

A

15-65 years

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31
Q

M or F for autoimmune diseases

A

F

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32
Q

percentage of the population with an autoimmune disease?

A

3%

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33
Q

molecules that enable laminar blood flow

A

heparin, TFPI, thrombomodulin, NO, prostacyclin

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34
Q

platelet cell surface receptors are for

A

ADP, epinephrine, thrombin

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35
Q

platelet alpha granule releases

A

VWF and thrombin

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36
Q

dense platelet granule releases

A

ADP, calcium, serotonin

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37
Q

clopidogrel works on the

A

ADP receptor

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38
Q

extrinsic pathway for clotting

A

Factor 7 and Tissue factor to factor 10-> prothrombin->thrombin which cleaves fibrinogen to fibrin

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39
Q

intrinsic pathway for clotting is

A

factor 11->9->8->10-> PT-> loads of thrombin = loads of fibrin

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40
Q

natural anticoagulants

A

protein C and S, anti-thrombin, tissue factor pathway inhibitor

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41
Q

Abciximab pathway of anticoagulation

A

GP IIb/IIIa

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42
Q

warfarin pathway of anticoagulation

A

9, 7, 10, prothrombin

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43
Q

Vit k dependent factors of coagulation

A

9, 7, 10

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44
Q

heparin mechanism of anticoagulation

A

10 and thrombin

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45
Q

Rivaroxaban mechanism of anticoagulation

A

ten

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46
Q

haematological malignancy M or F

A

M

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47
Q

haptens are

A

small molecule irritants that bind to proteins to elleicit an immune response

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48
Q

most common cause of an allergen is

A

peanut

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49
Q

percentage of the population effected by allergies

A

40%

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50
Q

polymorphism of what gene is responsible for eczema

A

filaggrin

51
Q

role of Mannan binding Lectin is

A

a collagen-like protein that binds sugars in bacterial cell walls and activates the classic complement pathway

52
Q

for gene therapy to be successful what criteria must be met?

A

evidence correcting mutation will improve condition, transfected gene will confer survival advantage and no cause malignancy

53
Q

immunodeficiency presents as SPUR

A

serious, persistent, unseals and recurrent infections

54
Q

MHC 2 is essential for

A

CD4+ T cell receptor

55
Q

MHC class 1 for essential for

A

CD8 T cells

56
Q

NK cells produce what for recruitment

A

IFN - gamma which stimulates macrophages, TH1 and CD8 cytotoxic T cells

57
Q

NK cells release what for apoptosis

A

perforin and granzyme

58
Q

which Ab is a pentamer

A

IgM

59
Q

which Ab is a dimer

A

IgA

60
Q

lectin, classical and alternative pathways all activate which complement protein?

A

C3

61
Q

C3 activating C3b leads too

A

membrane attack complex and opsonin

62
Q

C3 activating C3a leads too

A

inflammation

63
Q

membrane attack complex pathway

A

C3->C3b->C5->C5b->6,7,8,9,9,9,9 -> membrane attack complex

64
Q

complement proteins for chemotaxis are

A

C3a, C5a

65
Q

complement proteins for opsonisation are

A

C3b, C4b

66
Q

maintain solubility of Ab complexes complement proteins

A

C3b, C4b, C2

67
Q

examples of direct immunotherapy

A

monoclonal Ab, CARS, Bi-specific

68
Q

examples of indirect immunotherapy

A

tumour and dendritic cell vaccines, adoptive cell transfer, cytokine therapies, checkpoint inhibitor therapies, stimulatory antibodies

69
Q

hybridoma consists of

A

myeloma cell and the spleen cells from a mouse immunised with known antigen

70
Q

Fc portion of an antibody is defined by

A

the heavy chains

71
Q

5 types of heavy chain are

A 
IgG
IgA
Mu IgM
IgD
epsilon IgE
72
Q

most prevalent antibody subclass is

A

IgG

73
Q

light chains are either

A

Kappa or Lambda random selection for each cell

74
Q

Fab region of an antibody is

A

variable, defines bidnign

75
Q

IgG total levels

A

6-15g/L

76
Q

IgA total levels

A

1-4.5G/L

77
Q

IgM total levels

A

0.5-2.0g/L

78
Q

peak for myeloma

A

7th decade

79
Q

myeloma CRAB

A

hypercalcaemia, Renal failure, anaemia, Bone disease

80
Q

what percentage of bone marrow must be plasma cell for it to be myeloma

A

10%

81
Q

high risk neutropenia

A

<0.2x10*9/l

82
Q

significant risk neutropenia

A

<0.5x10*9/l

83
Q

time frame for high risk neutropenia

A

> 7 days

84
Q

additional risk factors for infection

A

disrupted skin, altered flora, lymphopenia, monocytopenia

85
Q

percentage of febrile neutropenia caused by gram positive bacteria

A

60-70%

86
Q

percentage of febrile neutropenia caused by gram negative bacteria

A

30-40%

87
Q

percentage of blasts in a bone biopsy of acute myeloblastic leukaemia

A

> 20% blasts of marrow cells

88
Q

acute promyelocytic leukaemia use of

A

all-trans retinoic acid and arsenic trioxide

89
Q

mode of action for a TKI inhibitor of BCR-ABL in chronic myelocytic leukaemia

A

imatinib blocks the ATP of BRC-ABL preventing the phosphorylation of tyrosine

90
Q

percentage of JAK2 mutations in polycythaemia Vera

A

95%

91
Q

acute lymphoblastic leukaemia most likely to occur in

A

children <6 years (75%)

92
Q

CD34, TDT are markers of what?

A

early immature cells

93
Q

CAR stands for

A

chimeric antigen receptor

94
Q

acute lymphoblastic leukaemia poor risk factors

A

T(9;22), slow response, increased WCC, increasing age

95
Q

chronic lymphocytic lymphoma stage A

A

<3 lymph node areas

96
Q

chronic lymphocytic lymphoma stage B

A

3 or more lymph node areas (8yr survival)

97
Q

chronic lymphocytic lymphoma stage C

A

anaemia and B symptoms or thrombocytopenia (6yrs)

98
Q

indications for treatment for chronic lymphocytic lymphoma

A

massive lymphadenopathy, splenomegaly, progressive marrow failure, lymphocyte doubling time <6 months, systemic symptoms

99
Q

commonest subtype of lymphoma

A

Diffuse large B Cell lymphoma a

100
Q

diffuse large B cell lymphoma high grade or low grade

A

high grade

101
Q

follicular lymphoma high grade or low grade?

A

low

102
Q

Hodgkin lymphoma is associated with

A

EBV

103
Q

hodgkin lymphoma constitutes what percentage of all lymphomas?

A

30%

104
Q

total body iron in approx.

A

4g

105
Q

iron is absorbed in the

A

duodenum

106
Q

immune haemolysis warm autoantibody causes

A

autoimmune, drugs, CLL

107
Q

immune haemolysis cold autoantibody causes

A

CHAD, infections, lymphoma

108
Q

immune haemolysis alloantibody causes

A

transfusion reaction

109
Q

intrinsic factor is secreted by

A

parietal cells

110
Q

B12 and intrinsic factor is absorbed In the

A

distal ileum

111
Q

other causes of microcytosis

A

alcohol, drugs, disordered liver function, hypothyroidism, myelodysplasia

112
Q

metals req. for RBC production

A

iron, copper, cobalt, manganese

113
Q

vitamins req. for RBC production

A

B12, folic acid, thiamine, Vit B6, amino acids

114
Q

hormones required for RBC production

A

erythropoietin, GM CSF, androgens, thyroxine, SCF

115
Q

G6PD transmission

A

X linked

116
Q

hair on end X-ray appearance indicates

A

Beta thalassaemia major

117
Q

Haemophilia A is a deficiency in which factor?

A

8

118
Q

haemophilia B is a deficiency in which factor?

A

9

119
Q

APTT measures which coagulation pathway?

A

intrinsic

120
Q

PT measures which coagulation pathway

A

extrinsic

121
Q

mother tells you father also have haemophilia (obligate carriers), chances of any other children having haemophilia;

A

1/4 chance

122
Q

will DDAVP work in severe haemophilia?

A

no only in mild

123
Q

normal platelet account is around

A

140-400

haemotology and immunology (3 decks)

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