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Phase 2a Introductory Clinical Science > Hematology > Flashcards

Hematology Flashcards

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1
Q

How much bone marrow does an adult have

A

2.5 kg

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2
Q

What are the two parts of the bone marrow and what do they have in them

A

red- hemopoietin cells

yellow- fatty cells

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3
Q

what changes occur to the bone marrow as you age

A

more yellow less red marrow

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4
Q

how many cells per day does the bone marrow make

A

500 million so loads of room for errors

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5
Q

What is leukaemia

A

malignant proliferation of hemopoietin cells

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6
Q

what are two common features of leukaemia

A

diffuse replacement of healthy bone marrow cells with leukaemic cells
variable amounts of abnormal cells in peripheral blood
accumulation of leukaemic cells in liver, spleen, lymph glands, meninges, gonads

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7
Q

what are the risk factors for leukaemia

A

congenital (downs increased risk)
chemotherapy
radiotherapy
benzene, Viruses e.g. HTLV and T cell leukaemia

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8
Q

What are the symptoms of leukaemia

A

anaemia with fatigue, SOB, hepatosplenomegaly
thrombocytopenia with easy bruising, purpuric rash, mucosal bleeding
infections with fevers and rigours

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9
Q

What is the rate of onset of symptoms of leukaemia

A

sudden usually

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10
Q

what investigations do you carry out for leukaemia

A

full blood count (low RBC, low platelets, high WBC, low mature neutrophils)
biochemistry- Urea, Calcium, folate, vitamin B12
chest XR
blood film (seeing blasts, rouleux for leukaemia)
flow cytometry to see antigens on cells
virology for hep B and C and HIV
coagulation test
cytogenic analysis for genetic abnormality
bone biopsy

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11
Q

What are the two forms of treatment for leukaemia

A

supportive and definitive

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12
Q

What do we offer in supportive care for leukaemia

A

bone marrow support via blood transfusion and platelets
antibiotic prophylaxis and anti-fungal prophylaxis
treat symptoms e.g. nausea, constipation from treatment
psychological support

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13
Q

What definitive treatment do we offer for leukaemia if intensive treatment

A

get into remission stage: high dose chemotherapy

stay in remission stage: stem cell transplant so graft cells attack cancer cells or chemotherapy for long time

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14
Q

What definitive treatment do we offer for leukaemia if palliative treatment

A

low dose chemotherapy to control disease

consider quantity of life vs quality of life

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15
Q

Describe what happens to leukaemic cells in acute leukaemia

A

lots of proliferation but loss of ability to differentiate so accumulation of blast cells in bone marrow which is a sign of bone marrow failure

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16
Q

What is a clinical feature of bone marrow failure

A

accumulation of blast cells in bone marrow

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17
Q

What happens to the peripheral WBC, RBC, platelets in acute leukaemia

A

low rbc, low platelets

wbc can be high, normal, low

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18
Q

What is acute lymphoblastic leukaemia (ALL)

A

proliferation of the lymphoblast cells

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19
Q

How can we classify ALL

A

B and T lymphoblast

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20
Q

When does ALL become common in regards to age

A

2-4 children
15-24 teens and adults
elderly

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21
Q

Which form of ALL is more common in adults, teens and children

A

Adults and teens T ALL

Children B ALL

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22
Q

What is a risk factor specific for acute lymphoblastic leukaemia

A

Philadelphia chromosome abnormality

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23
Q

What proportion of adults with ALL have Philadelphia chromosome abnormality? what about children

A

20-30% of adults

2% of children

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24
Q

In what age group is ALL rarer but has a worse prognosis

A

adults

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25
Q

How long do male vs female adults need to be treated to maintain remission of the leukaemia

A

3 years for male

2 years for female

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26
Q

What is the commonest malignant cancer in children aged 2-4

A

ALL

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27
Q

What is acute myeloid leukaemia (AML)

A

proliferation of the myeloblast cells

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28
Q

What is the most common acute leukaemia in adults

A

AML

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29
Q

When does AML incidence increase

A

increases with age increase

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30
Q

What makes an adult more likely to develop AML

A

bone marrow disorders

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31
Q

Do children develop AML from primary or secondary causes more

A

primary AML

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32
Q

What specific thing should you check for in a blood film and why

A

auer rods which guide prognosis

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33
Q

What does chronic leukaemia do to the bone marrow cells

A

Proliferation of bone marrow cells without bone failure. i.e. cells can still differentiate

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34
Q

What is the treatment for chronic leukaemia

A

no cure unless bone marrow transplant

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35
Q

What is chronic myeloid leukaemia

A

proliferation of myeloid cells i.e. of neutrophils, eosinophils, basophils, which replace normal bone marrow cells

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36
Q

What is a risk factor for chronic myeloid leukaemia specifically

A

philadelphia chromosome abnormality

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37
Q

What is median survival from CML without treatment

A

5 years

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38
Q

What is chronic lymphocytic leukaemia

A

proliferation of B lymphocyte cells that are resistant to apoptosis

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39
Q

What is the median age of survival of CLL without treatment

A

25 years

commonly disease of elderly and patients die with it

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40
Q

What are lymphomas

A

proliferation of wbc in lymph nodes or other organs in the lymphatic system

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41
Q

Where can the lymphoma spread to

A

blood, spleen, liver, other

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42
Q

What can cause lymphomas

A 
Infections: HTLV, Epstein Barr virus, Helicobacter pylori
idiopathic
radiation
primary and secondary immunodeficiency
autoimmune disease
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43
Q

What are the symptoms of lymphomas

A

nodal or extra-nodal
compression syndrome
B symptoms: weight loss, night sweats, loss of appetite

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44
Q

Which lymph nodes are esp. effected by lymphomas

A

neck
axilla
groin

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45
Q

What could a lump around the neck, groin, axilla mean other than lymphoma

A

inflammation or infection lump
disease of underlying structures
embryonic remnant
a metastatic tumour from other site

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46
Q

How much weight must be lost to count as a symptom of lymphoma

A

10% of normal body weight lost in 6 months

unintentionally

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47
Q

If a tumour metastasises to a lymph node, is that called lymphoma

A

no

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48
Q

How do we diagnose lymphoma and what’s the name of the machine

A 
haemato-oncology diagnostic service integrates:
- blood film
- bone marrow sample
- lymph node biopsy
- cytogenetics e.g. karyotype
- immunophenotype finds proteins on cell surface
- PCR
MDT to stage and grade
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49
Q

Give an example of a lymphoma cell we can diagnose using immunophenotype

A

B lymphocyte has CD20 on surface
only cell to have this
target treatment to CD20

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50
Q

What do we look at when staging lymphomas

A

CT scans
Bloods
PET scan (looks at where lots of glucose taken in by cells) naturally highlighted in liver, kidney, bladder, brain

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51
Q

What do we look at when assessing a patient

A 
History and examination
FBC
Virology for HIV, Hep B and C
CXR
Echo
Performance status regarding treatment
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52
Q

What scores can you get for performance status regarding treatment

A

0- asymptomatic can do all activities
1- symptomatic, can walk, can do light work not strenuous
2- symptomatic, bedbound<50% of day, can walk but can’t do any work, can do self-care
3- symptoms, bed bound >50% of day, can do self-care
4- symptoms, bed bound always
5- dead

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53
Q

What is a complication of lymphoma

A

idiopathic thrombocytopenic purpura aka immune thrombocytopenia
the immune system attacks platelets and platelet number low (usually single figures)
patient bleeds and bruises easily

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54
Q

What are the types of lymphoma

A

Hodgkin’s and Non-Hodgkin’s

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55
Q

When are the peaks in incidence of Hodgkin’s lymphoma

A

adolescent
elderly
bimodal

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56
Q

How does Hodgkin’s lymphoma present

A 
painless lymphadenopathy (abnormal size, number, constituent of lymph nodes)
B symptoms
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57
Q

How do we diagnose Hodgkin’s lymphoma

A

Reed-Sternberg cells presence

large lymphocytes that may have more than one nucleus

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58
Q

How many different histological subtypes of Hodgkin’s lymphoma are there

A

4

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59
Q

What are the stages of Hodgkin’s lymphoma

A 
numbers and letters (a= no symptoms, b= B symptoms)
1- one lymphoma at one site
2- two sites at same side of diaphragm
3- both sides of diaphragm affected
4- wide spread lymphoma
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60
Q

What chemotherapy do we use for Hodgkin’s Lymphoma

A 
ABVD
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
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61
Q

What treatment do we do first for stage 1-2a of H-Lymphoma

A

chemotherapy and radiotherapy

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62
Q

What treatment do we do first for stage 2b-4 of H-lymphoma

A

chemotherapy

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63
Q

What is the no-disease prolonged survival rate with treatment for stage 1-2a and stage 2b-4 of H-Lymphoma

A

stage 1-2a: 70-80%

stage 2b-4: 50-70%

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64
Q

What do we do if a patient relapses with H-lymphoma

A

autologous stem cell transplant
remove stem cells
high dose chemotherapy
re-introduce stem cells to build up bone marrow

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65
Q

What are the side effects of treatment of H-lymphoma

A 
infertility
heart, lung problems
peripheral neuropathy
psychological
other cancer risks from treatment and general susceptibility of patient
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66
Q

How can we reduce unnecessary high doses of treatments for H-lymphoma

A

PET scan

see which patients will respond well to treatment so give them lower doses

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67
Q

What are the three subtypes of Non-Hodgkin’s lymphoma

A

low, high , very high grade

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68
Q

give an example of low grade NH-Lymphoma

A

follicular lymphoma

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69
Q

What is presentation like for Follicular lymphoma

A

by time of presentation, it is usually advanced

its slow growing

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70
Q

What is the median time of survival for follicular lymphoma

A

9-11 years but can be longer or shorter for different patients

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71
Q

What are the treatment options for follicular lymphoma

A 
nothing (immune system handles for long time)
alkylating agents
bone marrow transplant
monoclonal antibodies
combined chemotherapy
radiotherapy
radio-immunoconjugate
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72
Q

Can we cure follicular lymphoma

A

no but can be treated

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73
Q

What is an example of a high grade NH-lymphoma

A

Diffuse large B cell lymphoma

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74
Q

Why is Diffuse Large B cell lymphoma also known as aggressive

A

If not treated it and cured, it will kill the patient

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75
Q

What is the progression of symptoms like in Diffuse Large B cell lymphoma

A

very quickly and severely unwell quickly

nodal presentation usually with 1/3 of patients having extra-nodal symptoms

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76
Q

What is early treatment of diffuse large B cell lymphoma

A

chemotherapy and radiotherapy

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77
Q

What is the advanced treatment of diffuse large B cell lymphoma

A

combined chemotherapy and monoclonal antibody

R-CHOP (Rituximab-(chemotherapy drugs CHOP)

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78
Q

What is Rituximab

A

monoclonal antibody
targets CD20 on B cells
Mouse and human protein
Mouse protein causes side effect: High BP, rash

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79
Q

Give an example of a very high NH-lymphoma

A

Burkitt’s lymphoma

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80
Q

What is myeloma

A

the malignant proliferation of plasma cells and clonal cells make one type of immunoglobulin

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81
Q

Give two plasma cell diseases associated with cancer that are not myelomas

A

plasmacytoma- solid tumour of plasma cells in/out of the bone marrow
Wederstorm’s Macroglobulinemia- to do with IgM

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82
Q

What are the progression steps before and during myeloma

A

Monoclonal gammopathy of undetermined significance (MGUS)
then Smouldering Myeloma then early myeloma
then late myeloma and then plasma cell leukaemia
during the timeline there are plateau (remission) phases and relapses

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83
Q

describe monoclonal gammopathy of undetermined significance (MGUS) and myeloma relationship

A

not a disease stage as there is no end organ damage here but it is a stage that gives potential for disease
1% per year risk of becoming myeloma
incidentally found with monoclonal immunoglobulin usually

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84
Q

relationship of smouldering myeloma and myeloma

A

significant risk of smouldering myeloma becoming myeloma

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85
Q

To make a diagnosis of myeloma you need…

A

clonal plasma cells (due to acquired chromosomal abnormality) and end-organ damage

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86
Q

What are the key end-organ damage seen in myeloma

A 
CRAB+/-
Calcium increase
Renal impairment
Anaemia
Bone disease
\+/- Infection, Amyloidosis (rare), Hyperviscosity (high protein in blood, rare)
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87
Q

What symptoms does the high calcium lead to in myeloma?

A

thirst, constipation, confusion

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88
Q

Why does the renal impairment occur in myeloma?

A

deposition of calcium and light chains from the immunoglobulins

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89
Q

What does the renal impairment in myeloma lead to?

A

dehydration

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90
Q

Why does the bone disease occur in myeloma?

A

increased osteoclast activity and decreased osteoblast activity
so more resorption
occurs mostly in weight-bearing bones

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91
Q

How does the high calcium occur in myeloma?

A

more resorption from bone disease so more calcium

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92
Q

What is a severe complication that can occur from myeloma due to the bone disease?

A

spinal cord compression

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93
Q

Why are there more infections in myeloma?

A

one type of immunoglobulin, not a variety to fight a variety of infections
also less maturation of other white blood cells e.g. neutrophils

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94
Q

What is a classical sign of amyloidosis?

A

big tongue

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95
Q

What kind of anaemia do you see in myeloma?

A

normocytic or macrocytic

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96
Q

Why does anaemia occur in myeloma?

A

less bone marrow space for maturation of red blood cells

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97
Q

What is the other key part of the blood decreased in myeloma?

A

less platelet production from bone marrow

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98
Q

What does low platelets cause in myeloma?

A

haematoma (subcutaneous bleeding)

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99
Q

What are some symptoms of myeloma?

A 
confusion
constipation
thirst
fatigue
bone pain esp. back
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100
Q

What are some signs of myeloma?

A 
anaemia
poor renal function
monoclonal protein in urine and protein
rouloux
high erythrocyte segmentation rate
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101
Q

What are the aims of treating myeloma?

A

control disease and induce remission
control symptoms
prophylaxis and supportive care

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102
Q

What treatment options do we use for myeloma?

A 
steroids
antivirals and antibiotics
biphosphates
combination chemotherapy
radiotherapy for specific lesions
Palliative care linked
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103
Q

What can cause spinal cord compression

A

Metastases and destabilisation of the spine due to tumours. commonly occurs in myeloma and plasmacytoma patients.

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104
Q

What are the symptoms of spinal cord compression

A 
back pain
lower leg weakness
saddle anesthesia
high anal tone
urinary retention: residual bladder volume >200ml
neuropathy
neurological damage means too late
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105
Q

What do we do if we have a patient with spinal cord compression

A

keep patient in bed
dexamethasone to shrink tumour
Urgent MRI to see if we need surgery or radiotherapy

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106
Q

Name two serious complications of myeloma

A

spinal cord compression

hyperviscosity syndrome

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107
Q

Why does hyperviscosity syndrome occur

A

malignant plasma cells produce lots of Ig which accumulate and as they are protein, raise the protein level in blood so becomes thick
the bigger the Ig the less needed to get this syndrome

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108
Q

What are the symptoms and signs of hyperviscosity syndrome

A 
bruising and mucosal bleeding
epistaxis
ataxia (loss of voluntary control of muscle movement)
nystagmus
blurred vision
heart failure signs: pulmonary oedema, fluid overload
SOB
headaches, confusion
fatigue
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109
Q

What is an easy examination to do when checking for hyperviscosity syndrome

A

check eyes

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110
Q

What is treatment for hyperviscosity syndrome

A

fluids via cannula

call haematologist

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111
Q

What is a negative side effect of chemotherapy which can lead to sudden death

A

tumour lysis syndrome

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112
Q

What is tumour lysis syndrome

A

when chemotherapy kills cancer cells, they release their intracellular components which are excreted by the kidneys so the kidneys are overwhelmed and crystallisation occurs. this and electrolyte imbalance leads to renal failure

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113
Q

What tumours are at risk particularly to tumour lysis syndrome

A

large tumours as more cells more death and components released
aggressive tumours as rapid turnover so more sensitive to chemo

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114
Q

What are the features of tumour lysis syndrome in the blood

A

hyperkalaemia
hypercalcemia
hyperphosphamia
hyperuremia

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115
Q

What are the clinical features of tumour lysis syndrome

A

renal failure
seizures
sudden death

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116
Q

How do you prevent tumour lysis syndrome

A

monitor patients that are at high risk with daily blood and urine checks for the electrolytes
vigours IV fluids
rasburicase to eliminate uric acid

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117
Q

How do we treat tumour lysis syndrome

A 
IV fluids
rasburicase
dialysis if necessary
treat high potassium, don't treat high calcium
monitor urine output
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118
Q

What are the reference ranges for hemoglobin, Mean cell volume of RBC, WBC, neutrophils, platelets

A 
hemoglobin: 131-166
MCV: 82-96
WBC: 3.5-9.5
neutrophils: 1.7-6.5
platelets: 150-400
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119
Q

How do we classify causes of too much of a substance in the blood

A

reactive/ secondary

proliferative/ primary

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120
Q

How do we classify causes of too little of a substance in the blood

A

under production

excess removal

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121
Q

What is too much red blood cells

A

polycythemia

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122
Q

What are the reactive causes of polycythemia

A

smoking
lung disease
androgen excess
altitude

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123
Q

What are the proliferative causes of polycythemia

A

polycythemia rubra vera

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124
Q

Why does polycythemia rubra vera occur

A

Myeloproliferative disorder of bone marrow

mainly red blood cells affected but also WBC, platelets

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125
Q

What is the genetic abnormality that is associated with polycythemia rubra vera

A

JAK2 mutation

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126
Q

What are the clinical signs of polycythemia rubra vera

A

thrombosis
itchy
red face
splenomegaly

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127
Q

How do we treat polycythemia rubra vera

A

venesection (remove blood)
aspirin
bone marrow suppression drugs e.g. hydroxycarbamide

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128
Q

What do we call too many wbc specifically of neutrophil type

A

neutrophilia

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129
Q

what are the reactive causes of neutriphilia

A

infection esp. bacterial
inflammation
trauma
malignancy

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130
Q

What is neutrophilia

A

too many WBC specifically too much neutrophils

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131
Q

What are the primary causes of neutrophilia

A

chronic myeloid leukaemia

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132
Q

What do we call too many WBC when neutrophils are normal

A

lymphocytosis

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133
Q

What is lymphocytosis

A

too much WBC, neutrophils normal though

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134
Q

What are the reactive causes of lymphocytosis

A

infection esp. virus
inflammation
trauma
malignancy

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135
Q

What are the primary causes of lymphocytosis

A

chronic lymphocytosis leukaemia

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136
Q

What do we call low neutrophils

A

neutropenia

137
Q

What does neutropenia mean

A

too low neutrophils

138
Q

What are the under-productive causes of neutropenia

A

bone marrow failure,
BM infiltration
BM toxicity e.g. from drugs

139
Q

What are the excess-removal causes of neutropenia

A

autoimmune disease
cyclical
felty syndrome

140
Q

How do we class severity of neutropenia

A

normal: 1.7-6.5
mild: 1-1.7
moderate: 0.5-1
severe: <0.5

141
Q

What are the clinical features of neutropenia sepsis

A 
rigours
fever
unwell
low blood pressure (rare)
cardiovascular collapse and sudden death
142
Q

Who is the patient that you always suspect neutropenia sepsis for?

A

patient that has had chemotherapy in last 3 months and has any of the clinical features of neutropenia sepsis

143
Q

How do we treat neutropenia

A

see patient

cannula and antibiotics: tazocin and gentamycin

144
Q

What do we call low platelets

A

thrombocytopenia

145
Q

What does thrombocytopenia mean

A

low platelets

146
Q

What do we call high platelets

A

thrombocytosis

147
Q

What does thrombocytosis mean

A

high platelets

148
Q

What are the reactive causes of thrombocytosis

A

infection
inflammation
malignancy

149
Q

What is a primary cause of thrombocytosis

A

essential thrombocythemia

150
Q

What does polycythemia mean

A

too much red blood cells

151
Q

What does polycythemia rubra vera cause

A

too much red blood cells

152
Q

What do we call low red blood cell mass

A

anaemia

153
Q

What does anaemia mean

A

low red blood cell mass +/- low hemoglobin concentration

154
Q

What are the consequences of anaemia

A

low oxygen transport-> tissue hypoxia-> compensatory physiologicla changes -> pathological changes

155
Q

What are the compensatory, physiological changes seen in anemia

A

increased perfusion - vasodilation, increased cardiac output
increased oxygen to tissue transfer
increased red blood cell production

156
Q

What are the pathological changes seen in anaemia

A 
fatty changes to myocardial tissue
fatty changes to liver
skin and nail atrophy
CNS cell death
aggregate angina conditions
157
Q

Where are red blood cells produced?

A

bone marrow

158
Q

How long do red blood cells survive in circulation

A

120 days

159
Q

Where are red blood cells removed

A

spleen, liver, bone marrow, blood loss

160
Q

How many red blood cells are removed

A

9 billion per day

161
Q

What are reticulocytes

A

immature red blood cells

162
Q

What is the reticulocyte test looking for

A

looks for immature red blood cells in blood to establish if there is an imbalance in production and removal of RBC

163
Q

What does a high and low reticulocyte number in the reticulocyte test mean?

A

high reticulocyte means increased removal as BM trying to produce lots of RBC doesn’t have time to mature them
low reticulocyte = increased production as BM maturing all the RBC

164
Q

How can anaemia be classified according to what causes it

A

production failures

increased cell loss, lysis or pooling

165
Q

What are causes of production failure anaemia

A

chronic disorders e.g. infection, inflammation, neoplasm
haematinic deficiency: iron b12, folic acid insufficient
hypo-plastic anaemia
bone marrow infiltration e.g. leukaemia and other metastatic neoplasms to bone marrow

166
Q

What are the causes of anaemia due to increased cell loss, pooling, lysis

A

acute blood loss

haemolytic anaemia

167
Q

How can we classify anaemia

A

size

what causes it

168
Q

How do we classify anaemia according to size

A

microcytic, normocytic, macrocytic

169
Q

What causes microcytic anaemia

A

iron deficiency
chronic diseases: renal, heart failure, cancer
thalassemia

170
Q

What is microcytic anaemia

A

small and pale RBC

171
Q

What is normocytic anaemia

A

normal sized RBC

172
Q

What causes normocytic anaemia

A

combined haematin deficiencies
chronic disease
Acute blood loss

173
Q

What is macrocytic anaemia

A

large and dark RBC

174
Q

what causes macrocytic anaemia

A

b12/folate deficiency
alcohol excess/ liver disease
hypothyroid
Haematological: bone marrow failure, bone marrow infiltration, antimetabolic therapy, haemolysis

175
Q

Is it better to have little or lots of iron

A

balance

iron is essential for formation of haem and thus RBC but too much causes deposits in myocardium, liver, pancreas

176
Q

How do we lose iron physiologically

A

sweat and cells lost in GU and GI system

uncontrolled loss

177
Q

How do we control iron levels

A

via absorption

we absorb 10% of dietary iron

178
Q

How much iron do we need in our diet

A

1mg men
1.5mg children
1.5-3 mg pregnant
2mg menstruating female

179
Q

Where is the iron in our body

A

60% in haemoglobin of RBC
30% in reticular-endothelial system
small % in muscle and iron-containing enzymes
small % in circulation

180
Q

What is ferritin

A

iron bound to it in reticular-endothelial system

protein-iron complex

181
Q

what form is the iron in during excess iron states

A

haemosiderin

182
Q

How much of transferrin is saturated with iron usually

A

1/3 so serum iron concentration is 1/3 of total-serum iron-binding capacity

183
Q

What are the causes of iron deficiency

A

chronic blood loss: peptic ulcers, cancers of rectum, colon
malabsorption
malnutrition
increased requirements e.g. childhood, pregnancy

184
Q

What do we investigate with iron deficiency

A

any causes to ongoing haemorrhage
heavy periods
breastfed only (breast milk low in iron)
hook worm infection check

185
Q

What are some symptoms of anaemia

A 
fatigue
SOB
palpitations
faintness
headache
186
Q

How would you treat iron deficient anaemia

A

treat underlying cause

iron tablets oral

187
Q

Why do we need vitamin B12

A

DNA synthesis and erythrocyte formation

188
Q

How do we absorb vitamin B12

A

parietal cells release intrinsic factor which binds to B12 and then the IF-B12 complex goes to iliac receptors and enters cells there.

189
Q

How much vitamin B12 is stored in the body

A

2.3mg

190
Q

How much vitamin B12 do we need per day

A

1ug

191
Q

Where in our diet do we get vitamin B12 from

A

yogurt, milk

usually, 20mg per day is enough

192
Q

Who is at risk of malnutritional causes of vitamin B12 deficiency

A

vegans

but would take years for it to show because stored amounts can keep us going for a long time

193
Q

What are the causes of vitamin B12 deficiency

A

pernicious anaemia
congenital lack of IF
gastroectomy causing lack of IF
crohn’s disease damage to ileum so lack of site of absorption
blind-looping syndrome due to bacteria competing for our B12
Ileac resectioning so loss of absorption site
malnutriiton-vegans

194
Q

What is pernicious anaemia

A

causes lack of IF
autoimmune causing atrophy of gastric mucosa with the failure of IF production due to antibody to the parietal cell
other antibodies against IF: one type stop B12 to IF and one type stops IF-B12 to ileac receptors

195
Q

What investigations to do we do when assessing B12 deficiency

A

antibodies against IF

Coeliac antibodies

196
Q

How do we treat vitamin B12 deficiency

A

Vit B12 replacement via IM injection

197
Q

Why do we need folic acid

A

DNA synthesis

198
Q

How much folic acid is stored in the body and where

A

liver mostly

10mg

199
Q

How much folic acid do we need per day

A

200ug

so deficiency can occur within weeks

200
Q

What can cause folic acid deficiency

A

malnutrition
malabsorption
increased demand for folate: pregnancy, malignancy
drugs e.g. some anti-cancer drugs are folic acid antagonists

201
Q

How do we investigate anaemia

A 
history and examination
FBC and blood film
reticulocyte count
folate, B12, ferritin investigations
TTP (red blood cell break down product from fibrin)
202
Q

What is hypoplastic anaemia

A

bone marrow and haematopoietic cells damaged

203
Q

What can cause hypoplastic anaemia

A

aplastic anaemia (inherited or acquired e.g. from radiation)
renal failure
pure red cell aplasia (bone marrow stops making red blood cells only)
endocrine dysfunction

204
Q

How can you divide causes of haemolytic anaemia

A

defects in and out of the cell

205
Q

What are causes of haemolytic anaemia that occur outside of the RBC

A 
immune haemolytic anaemia
microangiopathic haemolytic anaemia
burns
infections
hypersplenism
206
Q

What happens in immune haemolytic anaemia

A

autoantibodies or alloantibodies against foreign antigens, attack RBC

207
Q

What happens in microangiopathic haemolytic anaemia

A

RBC haemolysis occurs due to physical trauma when RBC pass through narrow or damaged vessels

208
Q

How do burns cause haemolytic anaemia

A

direct damage from heat to RBC in vessels in burned areas

burn damages the vessels so microangiopathic mechanism also

209
Q

What infection can cause haemolytic anaemia

A

malaria as the pathogens burst from the erythrocytes

210
Q

What are three types of defects inside the cell which lead to haemolytic anaemic

A

haemoglobinopathies
membranopathies
enzymopathies

211
Q

describe the globin chains in adult haemoglobin

A

2 alpha and 2 beta globin chains

low affinity to oxygen

212
Q

describe the globin chains in fetus haemoglobin

A

2 alpha and 2 gamma globin chains

high afinity to oxygen

213
Q

What are haemoglobinopathies

A

haemoglobinopathies refer to abnormal haemoglobin caused by single point mutation that causes amino acid substitution in the alpha or beta globin chains

214
Q

What are the two types of haemoglobinopathies and give an example for each

A

disorders of quality e.g. sickle cell disease

disorders of quantity e.g. thalassemia

215
Q

What is sickle cell disease

A

haemaoglobin S forms which has an abnormal beta globin chain due to single mutation on chromosome 11 from adenine to thymine leading to an amino acid substitution leaving valine in the beta chain. this causes the red blood cell to become sickle shaped.

216
Q

What does the abnormal beta chain in red blood cells lead to in sickle cell disease

A

the abnormal beta globin causes the haemoglobin to not dissolve but instead to crystalise in the red blood cells during low oxygen states. This damages the red blood cell and gives it a sickle shape

217
Q

What happens to the carriers of HbS

A

asymptomatic and protected against falciparum malaria

218
Q

Other than patients who are carriers of HbS, are there other patients with abnormal HbS who are protected against falciparum malaria

A

Combined heterozygotes with HbS and HbC

219
Q

What symptoms do patients with combined HbS and HbC show

A

milder chronic haemolytic anaemia
splenomegaly
no obstruction of microvascular

220
Q

What are the two main consequences of sickle cell disease

A

chronic haemolytic anaemia (RBC last 4-5days)

obstruction/occlusion of microvascular

221
Q

What are the main clinical features of sickle cell disease common in adults and children

A 
infections
chronic haemolytic anaemia
strokes
jaundice
splenic sequestration
splenomegaly
painful crisis
acute chest syndrome
222
Q

What are some clinical features of sickle cell disease common in adults mostly

A 
pulmonary hypertension
avascular necrosis
ocular disease
sickle neuropathy
leg ulcers
iron overload
223
Q

Which chromosome forms globin alpha, beta

A

alpha 16

beta 11

224
Q

What is the genotype for sickle cell disease to occur

A

HbS homozygous

Combined heterozygotes with HbS and HbC

225
Q

What are two acute complications of sickle cell disease

A

painful crisis

acute/sickle chest syndrome

226
Q

What are some chronic complications of sickle cell disease

A

renal failure
joint damage
pulmonary hypertension

227
Q

What causes painful crisis

A

cold weather, dehydration, infections precipitate it

228
Q

What is painful crisis

A

pain onset 2-3 days which is manageable at first and then becomes overwhelming in arms, legs, back, ribs, chest, abdomen

229
Q

How is painful crisis managed

A

analgesia and control pain

cannula fluids IV

230
Q

What is acute/sickle chest syndrome

A

tissue hypoxia leads to sickling leading to lung infarction and more hypoxia and more sickling
occurs with or after painful crisis often

231
Q

What are some features of sickle chest syndrome

A

XR change, hypoxia signs

232
Q

How do you treat sickle chest syndrome

A

oxygen, fluids, antibiotics

haemotologist will usually start exchange transfusion

233
Q

How do you treat sickle cell disease

A

exchange transfusion (dilates sickle cells but careful of iron overload)
hydroxycarbamide (reduces effects of sickle cells)
stem cell transplant

234
Q

How long do sickle red blood cells last for

A

4-5 days

235
Q

What is thalassemia

A

reduction in synthesis of alpha or beta globin chains so less haemoglobin. caused by mutation effecting expression of globin structural genes. Beta thalassemia less beta made and vice versa

236
Q

The damage caused by thalassemia: is that due to one or both globin chains

A

both
the globin chain that is not synthesised enough means that less haemolgobin can be made
the globin that is synthesised normally starts to accumulate and so damages erythrocytes maturing and mature.

237
Q

How can we classify thalassemia

A

according to if beta or alpha globin chain affected
according to clinical features:
major, intermedia, carrier

238
Q

Describe the clinical manifestations of thalassemia major, intermedia and carrier

A

major: needs transfusion for life
intermedia: less severe anaemia can survive without constant transfusion
carrier: asymptomatic

239
Q

How many alpha globin genes are there per red blood cell

A

4:

2 chromosome 16 and each has 2 alpha genes on them

240
Q

What do you call the condition if all the alpha globin genes are non-functioning
what are the symptoms

A

hydrops foetalis
not compatible with life
dies in utero

241
Q

What do you call the condition if 3 of the alpha globin genes are non-functioning
what are the symptoms

A

HbH disease
thalassemia syndrome
severe-moderate anaemia

242
Q

What do you call the condition if 2 of the alpha globin genes are non-functioning
what are the symptoms

A

alpha0 thalassemia trait

alpha+ thalassemia homozygote

243
Q

What do you call the condition if 1 of the alpha globin genes are non-functioning
what are the symptoms

A

alpha+ thalassemia trait

normal to minimum change

244
Q

What is the genetic description for alpha0 thalassemia trait

A

one of the chromosome 16 has no functioning alpha genes but the other one has both functioning alpha genes

245
Q

What is the genetic description for alpha+ thalassemia homozygote

A

each chromosome 16 has one functioning and one non-functioning alpha genes

246
Q

What is the genetic description for alpha+ thalassemia trait

A

one chromosome 16 has one non-functioning alpha gene and one functioning alpha gene
the other chromosome has both functioning alpha genes

247
Q

What is the genetic description for hydrops foetalis

A

no functioning alpha genes on either chromosome 16

248
Q

What can cause beta thalassemia

A

lots of genetic lesions

249
Q

What are the two types of beta thalassemia the genetic lesions cause

A

either no beta synthesis (beta0)

or restricted but a little beta synthesis (beta+)

250
Q

What is beta thalassemia major

A

severe thalassemia due to loss of two beta globin genes functioning

251
Q

When do patients usually present with beta thalassemia major

A

6-12months

252
Q

Why do patients usually present at 6-12 months with beta thalassemia major

A

that’s the time when fetal gamma globin should have been replaced by beta globin synthesis

253
Q

How do patients first present with beta thalassemia major

A

pale, crying, listless

failure to feed, growth retardation, poor sexual health (due to deposits in gonads and endocrine organs)

254
Q

What is the patient’s signs and clinical picture in beta thalassemia

A

severe microcytic and hypochromic anaemia
iron overload
liver enlargement
splenomegaly
red marrow expansion due to trying to make more red blood cells due to low haemoglobin but leads to bone thinning and bone growing on outer aspect

255
Q

How do we treat beta thalassemia major

A 
iron chelation
regular and lifelong transfusion
endocrine supplementation
bone health
psychological support
256
Q

What things in the long term do we monitor

A

endocrine monitor: diabetes, growth and puberty, gonad development
bone health: vitamin D, calcium, PTH
ferritin and haemosiderin
check heart and liver for iron deposits

257
Q

What are membranopathies

A

deficiencies of proteins in membranes of red blood cells that lead to abnormal membranes with red blood cells have less survival

258
Q

What are the two types of membranopathies

A

spherocytosis

elliptocytosis

259
Q

What interactions are damaged in spherocytosis

A

horizontal

260
Q

What interactions are damaged in elliptocytosis

A

vertical

261
Q

Which type of membranopathies are more common and more severe

A

spherocytosis

262
Q

Why are the RBC in spherocytosis patients survive for a short time

A

RBC leave bone marrow biconcave but due to abnormal membrane become spherical so get stuck in the microcirculation of spleen so spend longer time so are prematurely phagocytosis
also the spherical shape means the cell is more sensitive to osmotic stress

263
Q

What are two complications of membranopathies

A

jaundice

gallstones

264
Q

How can we treat membranopathies

A

folic acid supplements

splenectomy (heal anaemia fully)

265
Q

How bad is the haemolytic anaemia in patients with membranopathies

A

mild to moderate with occasional exacerbations e.g. due to infections

266
Q

What is enzymopathies

A

deficiency in enzymes needed for red blood cell survival to protect against oxidant compounds

267
Q

How do the enzymes in the red blood cell aid it

A

protect against oxidative compounds

fuel the red blood cell

268
Q

Give two examples of enzymopathies

A

glucose-6-phosphate dehydrogenase deficiency

pyruvate kinase deficiency

269
Q

What is glucose-6-phosphate dehydrogenase deficiency

A

low G6PD which usually reduces glutathione which protects RBC against oxidative damage

270
Q

Why is G6PD needed in the RBC

A

to protect haemoglobin and membrane against oxidative damage

271
Q

How is G6PD deficiency inherited

A

X linked inheritance

some women can be affected

272
Q

What symptoms does a person with G6PD deficiency experience

A

asymptomatic with crisis sometimes

crisis characterised by haemolysis, jaundice, anaemia

273
Q

What are treatment options for G6PD deficiency

A

crisis usually self-limiting

mostly avoid oxidative drugs, broad beans and infection which lead to crisis and more cell damage usually

274
Q

What drugs do you avoid with a person with G6PD deficiency

A 
quinolone
nitrofurantoin
primaquine
dapsone
sulphonamide
275
Q

How is pyruvate kinase deficiency inherited

A

autosomal recessive inheritence

276
Q

What is a virus that is particularly bad for a person with haemolytic anaemia

A

parvovirus

277
Q

What does parvovirus do the RBC

A

interferes with bone marrow so less produced for roughly a week
only serious if already have previous haemolytic anaemia as numbers of RBC suddenly drop

278
Q

What are the three phenotypes of anaemia that can protect against falciparum malaria

A

Carrier of HbS sickle cell
Patient with combined HbS and HbH
Carrier of G6PD deficiency gene

279
Q

How is membranopathies inherited

A

autosomal dominance

280
Q

What do we mean when we say disorders of blood coagulation and blood homeostasis

A

Blood coagulation- disorder of coagulation system

Blood homeostasis- disorder of platelets

281
Q

Where do platelets come from

A

bone marrow

megakaryocyte cytoplasm fragmentation

282
Q

What stimulates platelet formation

A

thrombocytopenia produced from the liver

283
Q

How is thrombocytopenia levels controlled

A

TPO binds to platelets and megakaryocytes
when platelets are low there is more free TP
so more free TPO binds to megakaryocytes and causes fragmentation so more platelets and more TPO bound and less free TPO

284
Q

Why can platelets flow close to the endothelium in vessels

A

disc shape

285
Q

Why do platelets have a disc shape

A

flow close to endothelium

286
Q

How many platelets do we make per megakaryocyte

A

4000

287
Q

How long do platelets live for

A

7-10 days

288
Q

Where are platelets removed

A

spleen by splenic macrophages

289
Q

What is platelets role in primary blood homeostasis

A

when exposed to collagen and vwf
platelets change shape and release granule content:
ADP, fibrinogen, calcium, thrombin
platelets aggregate by cross-linking with fibrin
form negatively charged layer that coagulation factors can bind to

290
Q

What are the important receptors on the surface of platelets

A 
thromboxane A2
P2Y12
Glycoprotein 2b3a, 1a, 1b
ABO
Human platelet antigen (HPA)
MHC class 1
291
Q

How is thromboxane A2 made

A

inside platelet by cyclooxygenase-1 (COX-1)

292
Q

What does thromboxaneA2 do

A

vasoconstriction

aggregate platelets

293
Q

What does P2Y12 get activated by

A

ADP

294
Q

What does P2Y12 do

A

activate GP receptors

activate platelets amplify

295
Q

What are platelet glycoprotein activated by and which receptors specifically

A

collagen (GP1a)

296
Q

What do platelet glycoprotein receptors do and which receptors specifically do this

A

bind to vwf and release dense and alpha granules

GP2B3A, 1B

297
Q

What kind of disorders can cause primary homeostasis disorders and which is more common

A

small vessel and platelet

platelet more common

298
Q

what investigations do you carry out for platelet disorders

A

FBC
Blood film
Platelet function assay (check function after giving aggregating agent)
flow cytometry

299
Q

What symptoms are there for platelet dysfunction

A

mucosal bleeding- nose, gums, meninges
easy bruising
purpura
traumatic haematoma

300
Q

What can cause bleeding

A 
injury
disorder of vascular 
platelet not functioning properly
low platelet number
coagulation disorder
301
Q

What is the difference between platelet disorder bleeding and coagulation disorder bleeding

A

coagulation disorder bleeding: spontaneous haematoma not traumatic like platelet dysfunction
rare purpura
rare mucosal bleeding unless urinary tract
joint bleeding common in severe
bleeding after surgery delayed not immediate like platelet dysfunction

302
Q

what can cause platelet dysfunctions

A

low number of platelets but normal function (thrombocytopenia)
platelets normal number but not functioning properly

303
Q

What can cause normal numbers but reduced function of platelets

A

medication e.g. aspirin
congenital abnormality
von Willibrand disease (low VWF activity)
urea

304
Q

What is thrombocytopenia

A

low platelets <150x10^9/L

caused by increased destruction or reduced production of platelets

305
Q

How low do the platelets have to get to cause increased traumatic bleeding

A

40-50x10^9

306
Q

How low do the platelets have to get to cause spontaneous skin and mucous bleeding

A

<20x10^9

307
Q

How can thrombocytopenia be classified

A

by what causes it

increase in destruction or decrease in production

308
Q

What are the causes of failure to produce platelets

A 
congenital- absent, low, dysfunctional megakaryocytes
infiltration of bone marrow
low production from bone marrow due to:
low folic acid/b12
low thrombopoietin e.g. from liver disease
toxin e.g. alcohol
medicine e.g. chemo
infections e.g. HIV, TB
309
Q

What are the causes of increased destruction of platelets

A

hypersplenism- high portal pressure, splenomegaly
autoimmune: primary, secondary immune thrombocytopenia
drug induced immune thrombocytopenia- e.g. heparin induced
consumption

310
Q

What does consumption mean in regards to platelets

A

platelets highly active so die quicker and production can’t keep up so low platelets

311
Q

What are the types of consumptions that cause thrombocytopenia

A

disseminating intravascular coagulopathy
thrombotic thrombocytopenic purpura
major haemorrhage
haemolytic uremic syndrome

312
Q

What occurs in immune thrombocytopenia

A

IgG bind to platelets so more opsonisation of platelets for spleen to destroy

313
Q

What are the differences between primary and secondary thrombocytopenia

A

primary after infection immunisation usually in children, acute
secondary, associated with infection e.g. HIV, malignancy, chronic

314
Q

What are the clinical signs of immune thrombocytopenia

A

haemorrhage, bruises, purpura

315
Q

How do we diagnose immune thrombocytopenia

A

exclude other causes

find underlying cause

316
Q

How do we treat immune thrombocytopenia

A

steroids
treat underlying cause
platelets (not long term because IgG will do same to new ones)
tranexamic acid to stop the breakdown of fibrin

317
Q

When must we not give tranexamic acid

A

urinary tract mucosal bleeding

318
Q

How does DIC occur

A

after sepsis, trauma, malignancy etc. cytokines released
activate systemic clotting cascade so consumption occurs
so less platelets so bleeding
so more activation of clotting cascade so thombosis in microvascular occurs and organ failure

319
Q

What are the main clinical signs for DIC

A

haemorrhage and signs of organ failure

320
Q

What do the DIC investigations show

A 
low platelets
high D-Dimers
low fibrinogen
high pro-thrombin time
\+/- organ failure
321
Q

How do we treat DIC

A

underlying cause
clotting factors
platelets
fibrinogen

322
Q

What is unusual about heparin-induced thrombocytopenia

A

causes thrombosis

323
Q

What happens in heparin-induced thrombocytopenia

A

heparin and platelet factor 4 form a complex that IgG binds to. This heparin-PF4-IgG complex activates platelets leading to over-activity and consumption

324
Q

What does heparin-induced thrombocytopenia cause

A

thrombosis

necrosis

325
Q

What is the presentation of heparin-induced thrombocytopenia

A

dramatic drop in platelet numbers 5-10 days after taking heparin

326
Q

Who is most at risk of heparin-induced thrombocytopenia

A

cardiac bypass recovery patients

patients on unfractionated heparin

327
Q

How do you treat heparin-induced thrombocytopenia

A

immediately stop heparin
give other anti-coagulation drugs
never give heparin again

328
Q

What is AMDAT13

A

VWF cleaving protease

metalloproteinase enzyme cleaves VWF

329
Q

Why does thrombotic thrombocytopenic purpura occur

A

immune system inhibits AMDATS13 so over-activity of platelets and consumption
causes thrombosis

330
Q

Where are thrombosis more likely to occur in thrombotic thrombocytopenic purpura

A

microvascular thrombosis esp in kidney, brain, heart

331
Q

How do you treat thrombotic thrombocytopenic purpura

A

plasma exchange to replace AMDATS13

immunosuppression medication

332
Q

Name 3 coagulation disorders all inherited

A

Haemophilia A
Haemophilia B
VWF deficiency

333
Q

Name one acquired condition that leads to coagulation disorders

A

vitamin K deficiency

334
Q

What is the difference between haemophilia A and B

A

A is a deficiency in factor 8

B is a deficiency in factor 9

335
Q

How are haemophilia A and B inherited

A

X linked recessive

336
Q

How are the genders affected by haemophilia A and B

A

Males affected

Female carriers have 50% of normal factor level

337
Q

How are haemophilia A and B treated

A

Clotting factor concentrates

338
Q

Where is VWF synthesised

A

Vascular endothelial cells

megakaryocytes

339
Q

How is VWF disease inherited

A

Autosomal dominance inheritence

Phase 2a Introductory Clinical Science (7 decks)

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