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
treatment for cold haemolytic anaemia
rituzamb
26
what is hereditary spherocytosis
- rather than being biconcave shaped it is shaped in a sphere (RBC) - affects people of European decent
27
what type of genetic condition is hereditary spherocytosis
autosomal dominant
28
why are RBCs in hereditary spherocytosis sphere shaped
genetic condition makes them have a problem in the cytoskeleton so they are not able to be biconcave
29
what happens in hereditary spherocytosis - why does it cause break down of RBCS
spleen doesn't accept these RBCs as they are shaped differently so it breaks them down
30
presentation of hereditary spherocytosis
children who are failing to thrive jaundice gallstones splenomegaly MCHC will be increased
31
what is MCHC
mean corpuscular hb concentration average conc of HB in a red blood cell
32
treatment of hereditary spherocytosis
acute - supportive treatment, transfusion long term treatment - folic treatment or removal of spleen
33
what would you seen under a microscope in hereditary spherocytosis
will be round no dip sphereocytes
34
what is sickle cell anaemia
- autosomal recessive - RBCs look sickle shaped - common in African people - can cause clots and infarcts - cells are fragile and breakdown
35
what is a sickle cell crisis
patient is hypoxic
36
pathophysiology of thalassemia
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
37
what is haemolytic disease of the new born
mother who is rhesus negative - her antibodies attack babies blood which is rhesus positive
38
what is pernicious anaemia
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
39
what is alpha thalassemia
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
40
alpha thalassemia - what do each of the mutations contribute towards
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
41
what is beta thalassemia
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
42
patient has headache, mouth ulcers, eye inflammation, skin rashes and blood vessels are inflamed throughout the body what is this
behcets disease
43
Immune thrombocytopenic purpura - what is it
when there is a low count of platelets results in easy bruising bleeding gums internal bleeding
44
what is the presentation of lead poisoning causing aneamia
absent relfexes increase in iron and ferritin muscle weakness
45
pernicious anaemia is?
autoantibodies attack the gastric parietal cells that produce a protein called intrinsic factor causes low vitamin b12 absorption
46
pernicious anaemia is common in which patients
patients who have other autoimmune conditions like thyroid disease and diabetes
47
what is chronic lymphocytic leukeamia
48
what are the 4 types of leukaemias
acute myeloid leukaemia (AML), acute lymphoblastic leukaemia (ALL), chronic lymphocytic leukaemia and chronic myeloid leukaemia.
49
difference between acute and chronic leukaemia
acute - due to impaired cell differentiation chronic - proliferation of malignant cells
50
myeloid vs lymphocytic leukaemia
myeloid - from myeloid precursor cells eg. neutrophils lymphocytic - B cells
51
acute myeloid leukaemia
most common in adults
52
what is the most common leukaemia in adults
acute myeloid leukaemia
53
features of AML
bone marrow failure anaemia bleeding infections organ swelling
54
diagnosing AML
low white cell count and AUER RODS
55
auer rods are found in the biopsy for what
AML
56
management of AML
bone marrow transplant chemo
57
acute lymphoblastic leukaemia ALL
- 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
features of ALL
fatigue abnormal bleeding infections bone pain painless lymphadenopathy cranial nerve palsies
59
diagnosis of ALL - what does blood results show
leukocytosis and blast cells
60
chronic myeloid leukaemia CML
- 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
features of CML
weight los tiredness fatigue fever sweating massive splenomegaly
62
what cells come under the myeloid lineage
neutrophils basophils eosinophils
63
what come under the lymphoid lineages
B cells T cells
64
what does the BCR-ABL gene mutation do
it causes the making of BCR-ABL protein this protein causes target cells to grow and divid out of control
65
3 phases of CML
chronic -> accelerated -> blastic
66
features of accelerated phase in CML
fever tiredness loss of appetite weight loss bruising night sweats bone pain changes in vision confusion tinnitus
67
blast phase of CML
cancer cells outgrow the healthy blood cells bone pain recurrent infections
68
diagnosis of CML
polymerase chain reaction test - shows the BCR-ABL protein ultrasound shows enlarged spleen
69
treatment of CML
tyrosine kinase inhibitors - bosutinib, dasatinib, imatinib
70
chronic lymphocytic leukaemia CLL - what is it
most common in male patients >60 abnormal B cells are made they crowd healthy cells
71
features of CLL
liver and spleen enlargement weight loss night sweats fevers bone marrow failure infection bleeding anaemia
72
diagnosis of CLL
smudge cells
73
CKD causes what change on the blood firm?
burr cells spiking of the RBC membrane
74
what is most the most common inherited thrombophilia
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
liver disease increases the risk of?
bleeding
76
what is Von Willebrand's disease
bleeding disorder - missing or defective clothing protein in the blood (von Willebrand factor) - excessive bleeding
77
what is the most common bleeding disorder
von willebrands disease
78
signs and symptoms of von willebrands disease
frequent and prolonged nose bleeds easy bruising excessive bleeding after surgery and dental work menorrhagia bleeding in joints
79
type 1 VWD
low levels of factor VWD and factor 8
80
type 2 VWD
body makes enough of factor VWD but it doesn't work in the way it should
81
type 3 VWD
makes very little or no VWD low levels of factor 8 most severe type
82
treatment of VWD
injections to cause clotting
83
what is acute chest crisis
- seen in patients with sickle cell anaemia - tachypnoea, wheeze, cough and hypoxia
84
management of acute chest crisis
high flow oxygen antibiotics urgent exchange transfusion
85
most common leukaemia in children?
ALL
86
most common leukaemia in adults?
AML/CML
87
what is intermittent porphyria
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
what are the features of CLL
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
management of immune thrombocytopenia pupura
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
what is Disseminated intravascular coagulation (DIC)
it is the innapproriate activation of the coagulation mechanism it causes low platelets and fibrogen
91
what is Thrombotic thrombocytopenic purpura
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
Immune thrombocytopaenia (ITP) what is it
is a type of platelet disorder. In ITP, your blood does not clot as it should, because you have a low platelet count.
93
Disseminated intravascular coagulation (DIC)
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
common presentation of VWD
heavy periods recurrent nose bleeds XS bleeding after tooth extraction desmopressin is given
95
side effects of repeated blood transfusions for beta thalassemia major
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
what is burrkitts lymphoma
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
what is polycaethemia
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
what is aplastic crisis
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
what is sequestration crisis
when there is pooling of blood in the spleen common in younger children present with shock sickle cell disease complication
100
what is acute chest syndrome
serious complication of sickle cell disease serious rest symptoms and fever wheeze and cough see pulmonary infiltrates
101
what is haemophilia A
it is a bleeding disorder that affects factor VIII levels
102
what type of cells are seen in blood film of AML
auer rods
103
what is the Richter formation?
when in CLL there is a rapid enlargement in lymph nodes
104