general Flashcards
1
Q
haemophilia A and B transmission
A
X-Linked
2
Q
risk of inheriting haemophilia A
A
1/10,000
3
Q
risk of inheriting haemophilia B
A
1/60,000
4
Q
haemophilia complications
A
viral infections, anti Factor 8 antibodies, MI with DDAVP
5
Q
epidemiology of von Willebrand disease
A
1/200
6
Q
transmission of Von Willebrand disease
A
autosomal
7
Q
type 1 von Willebrand disease refers too
A
quantitative deficiency
8
Q
type 3 von Willebrand disease refers too
A
complete deficiency
9
Q
3 examples of acquired bleeding disorders
A
liver failure, renal failure, warfarin
10
Q
Ecchymosis refers too
A
bruise
11
Q
ABO gene encodes for
A
glycosyltransferase
12
Q
A and B genes code for
A
transferase enzymes
13
Q
A antigen is
A
N-acetyl-galactosamine
14
Q
B antigen is
A
galactose
15
Q
out of A, B and O which is recessive and which is dominant?
A
A-B co dominant, O recessive
16
Q
blood donors screened for
A
Hep B, C, E, HIV, syphilis
17
Q
1 unit of RBC’s =
A
5g/L
18
Q
RBC’s transfusion time
A
2-4 hours
19
Q
shelf life of platelets
A
7 days
20
Q
Fresh frozen plasma for a bleeding patient with factor 8 or 10 deficiency
A
no concentrate is available
21
Q
Fresh frozen plasma for a bleeding patient on warfarin
A
no concentrate and Vit K
22
Q
bleeding patient on DOAC use fresh frozen platelets?
A
no
23
Q
a patient with DIC use fresh frozen platelets?
A
no
24
Q
bleeding patient with DIC use fresh frozen platelets?
A
yes
25
patient with massive haemorrhage but no coagulation results use fresh frozen platelets?
yes
26
hypotensive patient with low albumin use fresh frozen platelets?
no
27
epitope spreading refers too
breakdown of self tolerance, the resulting inflammation releases further self peptides and thus more inflammation
28
if the AIRE gene is mutated what kind of tolerance can't take place?
central
29
AIRE gene refers too
Autoimmune Regulator
30
peak onset for an autoimmune disease is
15-65 years
31
M or F for autoimmune diseases
F
32
percentage of the population with an autoimmune disease?
3%
33
molecules that enable laminar blood flow
heparin, TFPI, thrombomodulin, NO, prostacyclin
34
platelet cell surface receptors are for
ADP, epinephrine, thrombin
35
platelet alpha granule releases
VWF and thrombin
36
dense platelet granule releases
ADP, calcium, serotonin
37
clopidogrel works on the
ADP receptor
38
extrinsic pathway for clotting
Factor 7 and Tissue factor to factor 10-> prothrombin->thrombin which cleaves fibrinogen to fibrin
39
intrinsic pathway for clotting is
factor 11->9->8->10-> PT-> loads of thrombin = loads of fibrin
40
natural anticoagulants
protein C and S, anti-thrombin, tissue factor pathway inhibitor
41
Abciximab pathway of anticoagulation
GP IIb/IIIa
42
warfarin pathway of anticoagulation
9, 7, 10, prothrombin
43
Vit k dependent factors of coagulation
9, 7, 10
44
heparin mechanism of anticoagulation
10 and thrombin
45
Rivaroxaban mechanism of anticoagulation
ten
46
haematological malignancy M or F
M
47
haptens are
small molecule irritants that bind to proteins to elleicit an immune response
48
most common cause of an allergen is
peanut
49
percentage of the population effected by allergies
40%
50
polymorphism of what gene is responsible for eczema
filaggrin
51
role of Mannan binding Lectin is
a collagen-like protein that binds sugars in bacterial cell walls and activates the classic complement pathway
52
for gene therapy to be successful what criteria must be met?
evidence correcting mutation will improve condition, transfected gene will confer survival advantage and no cause malignancy
53
immunodeficiency presents as SPUR
serious, persistent, unseals and recurrent infections
54
MHC 2 is essential for
CD4+ T cell receptor
55
MHC class 1 for essential for
CD8 T cells
56
NK cells produce what for recruitment
IFN - gamma which stimulates macrophages, TH1 and CD8 cytotoxic T cells
57
NK cells release what for apoptosis
perforin and granzyme
58
which Ab is a pentamer
IgM
59
which Ab is a dimer
IgA
60
lectin, classical and alternative pathways all activate which complement protein?
C3
61
C3 activating C3b leads too
membrane attack complex and opsonin
62
C3 activating C3a leads too
inflammation
63
membrane attack complex pathway
C3->C3b->C5->C5b->6,7,8,9,9,9,9 -> membrane attack complex
64
complement proteins for chemotaxis are
C3a, C5a
65
complement proteins for opsonisation are
C3b, C4b
66
maintain solubility of Ab complexes complement proteins
C3b, C4b, C2
67
examples of direct immunotherapy
monoclonal Ab, CARS, Bi-specific
68
examples of indirect immunotherapy
tumour and dendritic cell vaccines, adoptive cell transfer, cytokine therapies, checkpoint inhibitor therapies, stimulatory antibodies
69
hybridoma consists of
myeloma cell and the spleen cells from a mouse immunised with known antigen
70
Fc portion of an antibody is defined by
the heavy chains
71
5 types of heavy chain are
```
IgG
IgA
Mu IgM
IgD
epsilon IgE
```
72
most prevalent antibody subclass is
IgG
73
light chains are either
Kappa or Lambda random selection for each cell
74
Fab region of an antibody is
variable, defines bidnign
75
IgG total levels
6-15g/L
76
IgA total levels
1-4.5G/L
77
IgM total levels
0.5-2.0g/L
78
peak for myeloma
7th decade
79
myeloma CRAB
hypercalcaemia, Renal failure, anaemia, Bone disease
80
what percentage of bone marrow must be plasma cell for it to be myeloma
10%
81
high risk neutropenia
<0.2x10*9/l
82
significant risk neutropenia
<0.5x10*9/l
83
time frame for high risk neutropenia
>7 days
84
additional risk factors for infection
disrupted skin, altered flora, lymphopenia, monocytopenia
85
percentage of febrile neutropenia caused by gram positive bacteria
60-70%
86
percentage of febrile neutropenia caused by gram negative bacteria
30-40%
87
percentage of blasts in a bone biopsy of acute myeloblastic leukaemia
>20% blasts of marrow cells
88
acute promyelocytic leukaemia use of
all-trans retinoic acid and arsenic trioxide
89
mode of action for a TKI inhibitor of BCR-ABL in chronic myelocytic leukaemia
imatinib blocks the ATP of BRC-ABL preventing the phosphorylation of tyrosine
90
percentage of JAK2 mutations in polycythaemia Vera
95%
91
acute lymphoblastic leukaemia most likely to occur in
children <6 years (75%)
92
CD34, TDT are markers of what?
early immature cells
93
CAR stands for
chimeric antigen receptor
94
acute lymphoblastic leukaemia poor risk factors
T(9;22), slow response, increased WCC, increasing age
95
chronic lymphocytic lymphoma stage A
<3 lymph node areas
96
chronic lymphocytic lymphoma stage B
3 or more lymph node areas (8yr survival)
97
chronic lymphocytic lymphoma stage C
anaemia and B symptoms or thrombocytopenia (6yrs)
98
indications for treatment for chronic lymphocytic lymphoma
massive lymphadenopathy, splenomegaly, progressive marrow failure, lymphocyte doubling time <6 months, systemic symptoms
99
commonest subtype of lymphoma
Diffuse large B Cell lymphoma a
100
diffuse large B cell lymphoma high grade or low grade
high grade
101
follicular lymphoma high grade or low grade?
low
102
Hodgkin lymphoma is associated with
EBV
103
hodgkin lymphoma constitutes what percentage of all lymphomas?
30%
104
total body iron in approx.
4g
105
iron is absorbed in the
duodenum
106
immune haemolysis warm autoantibody causes
autoimmune, drugs, CLL
107
immune haemolysis cold autoantibody causes
CHAD, infections, lymphoma
108
immune haemolysis alloantibody causes
transfusion reaction
109
intrinsic factor is secreted by
parietal cells
110
B12 and intrinsic factor is absorbed In the
distal ileum
111
other causes of microcytosis
alcohol, drugs, disordered liver function, hypothyroidism, myelodysplasia
112
metals req. for RBC production
iron, copper, cobalt, manganese
113
vitamins req. for RBC production
B12, folic acid, thiamine, Vit B6, amino acids
114
hormones required for RBC production
erythropoietin, GM CSF, androgens, thyroxine, SCF
115
G6PD transmission
X linked
116
hair on end X-ray appearance indicates
Beta thalassaemia major
117
Haemophilia A is a deficiency in which factor?
8
118
haemophilia B is a deficiency in which factor?
9
119
APTT measures which coagulation pathway?
intrinsic
120
PT measures which coagulation pathway
extrinsic
121
mother tells you father also have haemophilia (obligate carriers), chances of any other children having haemophilia;
1/4 chance
122
will DDAVP work in severe haemophilia?
no only in mild
123
normal platelet account is around
140-400
