Haematology Flashcards

1
Q

normacytic anaemia - what is it

A

normal sized red blood cells if seen under the microscope

  • but there is a deficiency of the red blood cells overall
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2
Q

definition of anaemia

A

deficiency of red blood cells

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3
Q

what are the causes of normacytic anaemia

A

CKD
aplastic anaemia
haemolytic anaemia
acute blood loss

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4
Q

what is macrocytic anaemia

A

anaemia of red blood cells that are larger in size compared to normal as seen under the microscope

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5
Q

causes of macrocytic anaemia

A

due to a deficiency - eg vitamin B12 or folic acid

so if anyone has a lack of any of those vitamins then they have this type if anaemia

alcohol
liver disease
hypothyroidism
cytotoxic drugs
reticulocytosis - too many immature red blood cells

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6
Q

what is microcytic anaemia

A

when seen under the microscope the RBCs are too small

normal hb
MCV will be low

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7
Q

causes of microcytic anaemia

A

iron deficiency anaemia
thalasemmia
lead poisoning
anaemia of chronic disease

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8
Q

why would you test an elderly patient urgently who came in with new onset microcytic anaemia

A

to rule out malignancy

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9
Q

haemolytic anaemia - what is it

A

red blood cells are being broken down

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10
Q

symptoms of haemolytic anaemia

A

low hb
rbc size will be low
fatigue
headaches
muscle weakness
jaundice

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11
Q

what is the role of the haptoglobin

A

it binds to free hb in the blood

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12
Q

what is the pathophysiology of haemolytic anaemia

A
  • because in this type of anaemia red blood cells are broken down
  • hb disperses
  • haptoglobin binds to hb
  • in this type of anaemia, free haptoglobin levels will be low as it will have bonded to the hb
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13
Q

why are patients with haemolytic anaemia jaundiced

A

bilirubin is a by product of blood when it is broken down
as RBCs are being broken down - more bilirubin will be present
so patient will be jaundiced

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14
Q

what are the urine results of someone who has haemolytic anaemia

A

raised hb in the urine

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15
Q

what are the different types of haemolytic anaemia

A

intravascular - is it happening within the vessel
extravascular - outside of the vessel

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16
Q

what is a common cause of intravascular haemolytic anaemia

A

G6PD deficiency (X linked condition) - common in African or Turkish, Arab or greek

enzyme doesn’t work properly, increases the likelihood of the RBC of undergoing oxidative stress
- more likely to break down

more likely to present in an acute setting
eating broad beans can cause this

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17
Q

other causes of intravascular haemolytic anaemia

A

drugs - antimalarials
mechanical heart valve - pressure of it can cause the RBCs to break down
cold haemolytic anaemiaa
mismatched blood transfusion

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18
Q

common features of intravascular haemolytic anaemia

A

neonatal jaundice

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19
Q

if someone has haemolytic anaemia what will you see under the microscope

A

Heinz bodies

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20
Q

what is cold auto immune haemolytic anaemia

or called cold agglutinin haemolytic anaemia

A

auto immune disease
own immune system attacks RBCs
happens if person is exposed to extreme cold temperatures
IgM antibodies - attack RBCs and they break down prematurely

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21
Q

causes of extravascular haemolytic anaemia

A

warm haemolytic anaemia
hereditary spherocytosis
sickle cell anaemia
thalassemia
haemolytic disease of the newborn

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22
Q

what is warm haemolytic anaemia

A

auto immune condition
happens if person is exposed to warm temperatures 37 degrees or above
IgG antibodies -attack RBC and they break down

can happen from body temperature

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23
Q

treatment for warm haemolytic anaemia

A

corticosteroids

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24
Q

what is the treatment for most auto immune diseases

A

steroids

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25
Q

treatment for cold haemolytic anaemia

A

rituzamb

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26
Q

what is hereditary spherocytosis

A
  • rather than being biconcave shaped it is shaped in a sphere (RBC)
  • affects people of European decent
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27
Q

what type of genetic condition is hereditary spherocytosis

A

autosomal dominant

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28
Q

why are RBCs in hereditary spherocytosis sphere shaped

A

genetic condition makes them have a problem in the cytoskeleton so they are not able to be biconcave

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29
Q

what happens in hereditary spherocytosis - why does it cause break down of RBCS

A

spleen doesn’t accept these RBCs as they are shaped differently
so it breaks them down

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30
Q

presentation of hereditary spherocytosis

A

children who are failing to thrive
jaundice
gallstones
splenomegaly
MCHC will be increased

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31
Q

what is MCHC

A

mean corpuscular hb concentration
average conc of HB in a red blood cell

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32
Q

treatment of hereditary spherocytosis

A

acute - supportive treatment, transfusion
long term treatment - folic treatment or removal of spleen

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33
Q

what would you seen under a microscope in hereditary spherocytosis

A

will be round
no dip
sphereocytes

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34
Q

what is sickle cell anaemia

A
  • autosomal recessive
  • RBCs look sickle shaped
  • common in African people
  • can cause clots and infarcts
  • cells are fragile and breakdown
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35
Q

what is a sickle cell crisis

A

patient is hypoxic

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36
Q

pathophysiology of thalassemia

A

hb is made of haem iron group in the centre and 4 global chains
2 alpha chains
2 beta chains
DNA mutations cause a reduction in the number and ability of RBCs to carry oxygen

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37
Q

what is haemolytic disease of the new born

A

mother who is rhesus negative - her antibodies attack babies blood which is rhesus positive

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38
Q

what is pernicious anaemia

A

auto immune condition that affects stomach
immune system attacks parietal cells in the stomach that produce a protein called an intrinsic factor

this helps vitamin b12 be absorbed into the blood

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39
Q

what is alpha thalassemia

A

not enough synthesis of alpha gains
excess chains of beta
there are 4 genes on the chromosome 16 that contributes towards the alpha region on hb
2 of the genes comes from each parent

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40
Q

alpha thalassemia - what do each of the mutations contribute towards

A

one mutation - no signs, silent carrier
2 mutations - mild signs, referred to as a trait
3 mutations - moderate or severe symptoms
4 symptoms - foetal death

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41
Q

what is beta thalassemia

A

not enough synthesis of beta chains
excess of alpha
2 genes on chromosome 11 that makes the beta chains
each one comes from each parent

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42
Q

patient has headache, mouth ulcers, eye inflammation, skin rashes and blood vessels are inflamed throughout the body

what is this

A

behcets disease

43
Q

Immune thrombocytopenic purpura - what is it

A

when there is a low count of platelets
results in easy bruising
bleeding gums
internal bleeding

44
Q

what is the presentation of lead poisoning causing aneamia

A

absent relfexes
increase in iron and ferritin
muscle weakness

45
Q

pernicious anaemia is?

A

autoantibodies attack the gastric parietal cells that produce a protein called intrinsic factor

causes low vitamin b12 absorption

46
Q

pernicious anaemia is common in which patients

A

patients who have other autoimmune conditions like thyroid disease and diabetes

47
Q

what is chronic lymphocytic leukeamia

A
48
Q

what are the 4 types of leukaemias

A

acute myeloid leukaemia (AML),
acute lymphoblastic leukaemia (ALL),
chronic lymphocytic leukaemia and
chronic myeloid leukaemia.

49
Q

difference between acute and chronic leukaemia

A

acute - due to impaired cell differentiation
chronic - proliferation of malignant cells

50
Q

myeloid vs lymphocytic leukaemia

A

myeloid - from myeloid precursor cells eg. neutrophils
lymphocytic - B cells

51
Q

acute myeloid leukaemia

A

most common in adults

52
Q

what is the most common leukaemia in adults

A

acute myeloid leukaemia

53
Q

features of AML

A

bone marrow failure
anaemia
bleeding
infections
organ swelling

54
Q

diagnosing AML

A

low white cell count
and AUER RODS

55
Q

auer rods are found in the biopsy for what

A

AML

56
Q

management of AML

A

bone marrow transplant
chemo

57
Q

acute lymphoblastic leukaemia ALL

A
  • most common cancer of childhood
  • bone marrow makes too many lymphocytes which are abnormal so don’t work properly
    these crowd function of the healthy cells
  • can cause infection, anaemia and bleeding
58
Q

features of ALL

A

fatigue
abnormal bleeding
infections
bone pain
painless lymphadenopathy
cranial nerve palsies

59
Q

diagnosis of ALL - what does blood results show

A

leukocytosis and blast cells

60
Q

chronic myeloid leukaemia CML

A
  • middle aged patients
  • it is of the small, mature stem cells that are dysfunctional
  • caused by mutation which causes formation of BCR-ABL gene
61
Q

features of CML

A

weight los
tiredness
fatigue
fever
sweating
massive splenomegaly

62
Q

what cells come under the myeloid lineage

A

neutrophils
basophils
eosinophils

63
Q

what come under the lymphoid lineages

A

B cells
T cells

64
Q

what does the BCR-ABL gene mutation do

A

it causes the making of BCR-ABL protein
this protein causes target cells to grow and divid out of control

65
Q

3 phases of CML

A

chronic -> accelerated -> blastic

66
Q

features of accelerated phase in CML

A

fever
tiredness
loss of appetite
weight loss
bruising
night sweats
bone pain
changes in vision
confusion
tinnitus

67
Q

blast phase of CML

A

cancer cells outgrow the healthy blood cells
bone pain
recurrent infections

68
Q

diagnosis of CML

A

polymerase chain reaction test - shows the BCR-ABL protein
ultrasound shows enlarged spleen

69
Q

treatment of CML

A

tyrosine kinase inhibitors - bosutinib, dasatinib, imatinib

70
Q

chronic lymphocytic leukaemia CLL - what is it

A

most common in male patients >60
abnormal B cells are made
they crowd healthy cells

71
Q

features of CLL

A

liver and spleen enlargement
weight loss
night sweats
fevers
bone marrow failure
infection
bleeding
anaemia

72
Q

diagnosis of CLL

A

smudge cells

73
Q

CKD causes what change on the blood firm?

A

burr cells

spiking of the RBC membrane

74
Q

what is most the most common inherited thrombophilia

A

factor V leiden
increases risk of Venous thromboembolism
- point mutation in factor V
- this factor is NOT broken down by activated protein C
- thromboses forms

75
Q

liver disease increases the risk of?

A

bleeding

76
Q

what is Von Willebrand’s disease

A

bleeding disorder
- missing or defective clothing protein in the blood (von Willebrand factor)
- excessive bleeding

77
Q

what is the most common bleeding disorder

A

von willebrands disease

78
Q

signs and symptoms of von willebrands disease

A

frequent and prolonged nose bleeds
easy bruising
excessive bleeding after surgery and dental work
menorrhagia
bleeding in joints

79
Q

type 1 VWD

A

low levels of factor VWD and factor 8

80
Q

type 2 VWD

A

body makes enough of factor VWD but it doesn’t work in the way it should

81
Q

type 3 VWD

A

makes very little or no VWD
low levels of factor 8

most severe type

82
Q

treatment of VWD

A

injections to cause clotting

83
Q

what is acute chest crisis

A
  • seen in patients with sickle cell anaemia
  • tachypnoea, wheeze, cough and hypoxia
84
Q

management of acute chest crisis

A

high flow oxygen
antibiotics
urgent exchange transfusion

85
Q

most common leukaemia in children?

A

ALL

86
Q

most common leukaemia in adults?

A

AML/CML

87
Q

what is intermittent porphyria

A

it is a genetic condition in which there is a difficulty in the synthesis of the haem group
it causes red-urine
and shaking
abdo pain
low mood

a mix of symptoms

88
Q

what are the features of CLL

A

CLL is due to the monoclonal proliferation of B-lymphocytes
they present the antigen CD20
when preparing the blood film, these cells can easily break making
SMUDGE CELLS

89
Q

management of immune thrombocytopenia pupura

A

it is when the immune system attacks the platelets and breaks them down
if platelets are less than 30 then do oral prednisolone therapy

this suppresses the immune system and stops them from breaking down the platelets

90
Q

what is Disseminated intravascular coagulation (DIC)

A

it is the innapproriate activation of the coagulation mechanism
it causes low platelets and fibrogen

91
Q

what is Thrombotic thrombocytopenic purpura

A

a rare, life-threatening blood disorder. In TTP, blood clots form in small blood vessels throughout your body. The clots can limit or block the flow of blood to your organs, such as your brain, kidneys, and heart. This can prevent your organs from working properly and can damage your organs.

92
Q

Immune thrombocytopaenia (ITP) what is it

A

is a type of platelet disorder. In ITP, your blood does not clot as it should, because you have a low platelet count.

93
Q

Disseminated intravascular coagulation (DIC)

A

a rare but serious condition that causes abnormal blood clotting throughout the body’s blood vessels.

this then as a result causes more bleeding in other areas even after a slight injury
this is because the platelets have been used up in the wrong places

94
Q

common presentation of VWD

A

heavy periods
recurrent nose bleeds
XS bleeding after tooth extraction

desmopressin is given

95
Q

side effects of repeated blood transfusions for beta thalassemia major

A

it can cause too much deposit of iron
can cause pitiutary failure
this can cause stopping of periods and leg swelling due to heart failure

it can also present with swelling in the joints and hyperpigmentation

give Desferrioxamine to clear out the iron

96
Q

what is burrkitts lymphoma

A

it is a more aggressive non-hodgkins lymphoma

has a ‘starry sky’ appearance on the blood firm
having had EBV is a risk factor

97
Q

what is polycaethemia

A

it is when there is a high level of hb and haemltocrit in the blood
caused by JAK2 gene mutation

itchiness after warm showers
red complexion of skin
more likely to get VTE

98
Q

what is aplastic crisis

A

it is a complication of sickle cell disease

sudden drop in bone marrow production and RBCs so HB levels decrease rapidly
generally happens due to B19 parvovirus infection

99
Q

what is sequestration crisis

A

when there is pooling of blood in the spleen
common in younger children
present with shock

sickle cell disease complication

100
Q

what is acute chest syndrome

A

serious complication of sickle cell disease
serious rest symptoms and fever
wheeze and cough

see pulmonary infiltrates

101
Q

what is haemophilia A

A

it is a bleeding disorder that affects factor VIII levels

102
Q

what type of cells are seen in blood film of AML

A

auer rods

103
Q

what is the Richter formation?

A

when in CLL there is a rapid enlargement in lymph nodes

104
Q
A