Haematology Flashcards
normacytic anaemia - what is it
normal sized red blood cells if seen under the microscope
- but there is a deficiency of the red blood cells overall
definition of anaemia
deficiency of red blood cells
what are the causes of normacytic anaemia
CKD
aplastic anaemia
haemolytic anaemia
acute blood loss
what is macrocytic anaemia
anaemia of red blood cells that are larger in size compared to normal as seen under the microscope
causes of macrocytic anaemia
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
what is microcytic anaemia
when seen under the microscope the RBCs are too small
normal hb
MCV will be low
causes of microcytic anaemia
iron deficiency anaemia
thalasemmia
lead poisoning
anaemia of chronic disease
why would you test an elderly patient urgently who came in with new onset microcytic anaemia
to rule out malignancy
haemolytic anaemia - what is it
red blood cells are being broken down
symptoms of haemolytic anaemia
low hb
rbc size will be low
fatigue
headaches
muscle weakness
jaundice
what is the role of the haptoglobin
it binds to free hb in the blood
what is the pathophysiology of haemolytic anaemia
- 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
why are patients with haemolytic anaemia jaundiced
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
what are the urine results of someone who has haemolytic anaemia
raised hb in the urine
what are the different types of haemolytic anaemia
intravascular - is it happening within the vessel
extravascular - outside of the vessel
what is a common cause of intravascular haemolytic anaemia
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
other causes of intravascular haemolytic anaemia
drugs - antimalarials
mechanical heart valve - pressure of it can cause the RBCs to break down
cold haemolytic anaemiaa
mismatched blood transfusion
common features of intravascular haemolytic anaemia
neonatal jaundice
if someone has haemolytic anaemia what will you see under the microscope
Heinz bodies
what is cold auto immune haemolytic anaemia
or called cold agglutinin haemolytic anaemia
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
causes of extravascular haemolytic anaemia
warm haemolytic anaemia
hereditary spherocytosis
sickle cell anaemia
thalassemia
haemolytic disease of the newborn
what is warm haemolytic anaemia
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
treatment for warm haemolytic anaemia
corticosteroids
what is the treatment for most auto immune diseases
steroids
treatment for cold haemolytic anaemia
rituzamb
what is hereditary spherocytosis
- rather than being biconcave shaped it is shaped in a sphere (RBC)
- affects people of European decent
what type of genetic condition is hereditary spherocytosis
autosomal dominant
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
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
presentation of hereditary spherocytosis
children who are failing to thrive
jaundice
gallstones
splenomegaly
MCHC will be increased
what is MCHC
mean corpuscular hb concentration
average conc of HB in a red blood cell
treatment of hereditary spherocytosis
acute - supportive treatment, transfusion
long term treatment - folic treatment or removal of spleen
what would you seen under a microscope in hereditary spherocytosis
will be round
no dip
sphereocytes
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
what is a sickle cell crisis
patient is hypoxic
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
what is haemolytic disease of the new born
mother who is rhesus negative - her antibodies attack babies blood which is rhesus positive
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
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
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
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
patient has headache, mouth ulcers, eye inflammation, skin rashes and blood vessels are inflamed throughout the body
what is this
behcets disease
Immune thrombocytopenic purpura - what is it
when there is a low count of platelets
results in easy bruising
bleeding gums
internal bleeding
what is the presentation of lead poisoning causing aneamia
absent relfexes
increase in iron and ferritin
muscle weakness
pernicious anaemia is?
autoantibodies attack the gastric parietal cells that produce a protein called intrinsic factor
causes low vitamin b12 absorption
pernicious anaemia is common in which patients
patients who have other autoimmune conditions like thyroid disease and diabetes
what is chronic lymphocytic leukeamia
what are the 4 types of leukaemias
acute myeloid leukaemia (AML),
acute lymphoblastic leukaemia (ALL),
chronic lymphocytic leukaemia and
chronic myeloid leukaemia.
difference between acute and chronic leukaemia
acute - due to impaired cell differentiation
chronic - proliferation of malignant cells
myeloid vs lymphocytic leukaemia
myeloid - from myeloid precursor cells eg. neutrophils
lymphocytic - B cells
acute myeloid leukaemia
most common in adults
what is the most common leukaemia in adults
acute myeloid leukaemia
features of AML
bone marrow failure
anaemia
bleeding
infections
organ swelling
diagnosing AML
low white cell count
and AUER RODS
auer rods are found in the biopsy for what
AML
management of AML
bone marrow transplant
chemo
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
features of ALL
fatigue
abnormal bleeding
infections
bone pain
painless lymphadenopathy
cranial nerve palsies
diagnosis of ALL - what does blood results show
leukocytosis and blast cells
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
features of CML
weight los
tiredness
fatigue
fever
sweating
massive splenomegaly
what cells come under the myeloid lineage
neutrophils
basophils
eosinophils
what come under the lymphoid lineages
B cells
T cells
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
3 phases of CML
chronic -> accelerated -> blastic
features of accelerated phase in CML
fever
tiredness
loss of appetite
weight loss
bruising
night sweats
bone pain
changes in vision
confusion
tinnitus
blast phase of CML
cancer cells outgrow the healthy blood cells
bone pain
recurrent infections
diagnosis of CML
polymerase chain reaction test - shows the BCR-ABL protein
ultrasound shows enlarged spleen
treatment of CML
tyrosine kinase inhibitors - bosutinib, dasatinib, imatinib
chronic lymphocytic leukaemia CLL - what is it
most common in male patients >60
abnormal B cells are made
they crowd healthy cells
features of CLL
liver and spleen enlargement
weight loss
night sweats
fevers
bone marrow failure
infection
bleeding
anaemia
diagnosis of CLL
smudge cells
CKD causes what change on the blood firm?
burr cells
spiking of the RBC membrane
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
liver disease increases the risk of?
bleeding
what is Von Willebrand’s disease
bleeding disorder
- missing or defective clothing protein in the blood (von Willebrand factor)
- excessive bleeding
what is the most common bleeding disorder
von willebrands disease
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
type 1 VWD
low levels of factor VWD and factor 8
type 2 VWD
body makes enough of factor VWD but it doesn’t work in the way it should
type 3 VWD
makes very little or no VWD
low levels of factor 8
most severe type
treatment of VWD
injections to cause clotting
what is acute chest crisis
- seen in patients with sickle cell anaemia
- tachypnoea, wheeze, cough and hypoxia
management of acute chest crisis
high flow oxygen
antibiotics
urgent exchange transfusion
most common leukaemia in children?
ALL
most common leukaemia in adults?
AML/CML
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
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
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
what is Disseminated intravascular coagulation (DIC)
it is the innapproriate activation of the coagulation mechanism
it causes low platelets and fibrogen
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.
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.
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
common presentation of VWD
heavy periods
recurrent nose bleeds
XS bleeding after tooth extraction
desmopressin is given
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
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
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
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
what is sequestration crisis
when there is pooling of blood in the spleen
common in younger children
present with shock
sickle cell disease complication
what is acute chest syndrome
serious complication of sickle cell disease
serious rest symptoms and fever
wheeze and cough
see pulmonary infiltrates
what is haemophilia A
it is a bleeding disorder that affects factor VIII levels
what type of cells are seen in blood film of AML
auer rods
what is the Richter formation?
when in CLL there is a rapid enlargement in lymph nodes
