Haematology Flashcards
panyctopenia
all type of blood cells are low
define anaemia
low concentration of haemaglobin in the blood
(consequence of underlying disease and is NOT a disease in itself)
what is haemaglobin
protein found in RBCs - responsible for picking up oxygen in the lungs and transporting it to the bodys cells.
why is iron essiential?
helps make haemaglobin and forms part of its structure
what is the MCV: mean cell volume?
the size of RBCs
normal MCV in male/female
80-100 femtolitres
what is reticulocytosis
increased concentration of recticulocytes (immature RBC)
this happens when there is a rapid tunroenr of RBC (haemolytic anaemia or blood loss)
where is iron mainly absorbed
duodenum and jejnunum
(it requires acid from the stomach to keep iron in soluble Ferras - Fe2+ form), when the stomach acid is less acidic , it changes to ferric (Fe3+) form.
what commonly prescribed medication may interfere with iron absorption
PPIs!! - reduced stomach acid
stomach acid keeps iron in soluble form (Ferrous Fe2+)
what GI disorders interfere with iron absorbption
coeliac disease or crohns - because there is inflammation of duodenum and jejunum where iron is absorbed
how is iron transported around the body?
Transferrin (carrier protein)
what is total iron binding capacity (TIBC)
the space for iron to attach to on all the transferrin molecules combined - measurement is directly related tot he amount of transferrin in the blood.
what is transferrin saturation?
the proportion of transferrin molecules bound to iron (%)
Transferrin saturation = serum iron/ total bind capacity
what is ferrtin
a protein that stores iron inside cells
Ferrtin is an acute phase protein , released with inflammation (infection or cancer)
a. true
b. false
a. true
raised ferrtin can be caused by inflammation, liver disease, supplements, haemochromatosis
Normal ferrtin excludes iron deficiency
a. true
b. false
b. false
what is a marker for how much transferrin is in the blood?
total iron binding capacity
where is B12 absorbed?
the distal ileum
what protein is needed for the absorbption of vitamin b12 in the distal ileum?
instrinsic factor which is released by parietal cells in the stomach
which do you treat first if B12 and folate are low and why?
Treat B12 first
if you give folate first you can cause – subacute combined degeneratin of the cord (demyelination in the spinal cord and severe neurological problems)
what type of anaemia will coombs test be positive?
autoimmune* haemolytic anaemia
what is the MCV of most haemolytic anaemias?
normocytic
what is found on the blood film of someone with G6DP deficiency (enzyme that protects cells from oxidative stress)
heinz bodies
inheritance mechanism of G6DP deficiency
X-linked
at what point in Hb level would you consider blood transfusion
< 70 Hb
vital in haemolytic crisis
B12/folate deficiency are classed as what type of anaemias?
macrocytic - megaloblastic
causes of microcytic anaemia?
TAILS
Thalassaemia
Anaemia of chronic disease 30%
Iron deficiency
Lead poisoing
Siderblastic anaemia
causes of normocytic anaemia
3A, 2H
A- acute loss of blood (recent)
A- Anaemia of chronic disease (more so)
A- Aplastic Anaemia (reduced production)
H- Haemolytic Anaemia
H- Hypothyroidism
What could cause recticulocyte count to increase in anaemia?
- acute blood loss
- haemolysis
body responds by making more
(both normocytic)
what is megaloblastic anaemia the result of
impaired DNA synthesis - (preventing cells from dividing - they grow large and abnormally)
B12/folate deficiency
why is iron essiential to RBCs
it is a key component of hemoglobin, the protein in RBCs that carries oxygen throughout the body. Without enough iron, RBCs cannot function properly, leading to conditions like iron-deficiency anemia.
Iron is the main ingredient in heme, the oxygen-binding part of hemoglobin.
Each hemoglobin molecule contains four heme groups, each holding an iron atom that binds oxygen.
pica is common in children with which kind of anaemia
iron deficiency
signs of iron deficient anameia
hair loss
koilonychia (spoon shaped nails)
angular chelitis
atropic glossitis (smooth tongue due to atrophy of papillae
brittle hair and nails
what is ferritin
the protein that stores iron in the body and releases it when it is needed
(found in the liver, spleen, bone marrow and muscles)
what is the function of ferropotin
it helps move iron out of the cells and into the blood stream (it is found ont he surface of iron sotring cells like enterocytes in the gut, macrophages and hepatocytes in the liver) - helps release iron into the bloodstream
transferrin does what?
is the protein in blood that binds to iron and transports it to various tissues and organs (including bone marrow where RBC are produced)
critical in the regulation of iron distribution
when iron is low - transferrin may increase to where it is needed
when iron is high - transferrin is low (body doesnt need as much iron transport)
Ferrous Fe2+ is easier for the boy to absorb than Fe3+
a. true
b. false
a. true
Fe3+ is less soluble and is converted to Ferrous by the the sotmach acid or vitamin C before it can be absorbed (duodenum/jejunum)
What happens to iron once it is absorbed?
transported by transferrin- (shuttles iron) to cells where it can be absorbed (ferritin) until it is needed
(released and converted back to soluble form - used in RBC production)
what is hepcidin
hormone produced by the liver that binds to ferroportin and causes it to be degraded - reducing iron export when iron levels are too high.
What will MCV level be in iron deficient anaemia
LOW
- microcytic
hypochromic - pale cells due to reduced Hb concentration
what is the total iron binding capacity?
space for iron to attach to on all transferrin moleules combined
(increases in low iron)
what is transferrin
carrier protein
- increases during iron deficiency anaemia (to try and pick up/transport more iron)
what is the transferrin saturation
amount of transferrin molecules bound to iron - serum iron/total iron binding capacity
will decrease
ferritin levels in iron deficient anaemia
ferriton low
- stores iron (iron stores low)
treatment for iron deficient anaemia
- oral - ferrous sulphate or fumarate
- iron infusion (IV cosmofer)
- blood transfusion (severe)
when not to give iron/iron infusions
infections - can feed the bacteria!!!
where is iron absorbed
duodenum/jejunum
(reduced in coeliac disease)
what is thalassaemia
abnormal synthesis of Hb
- reduced/faultyor absent globin chain production
both alpha and beta thalassaemia are autosomal recessive
a.true
b. false
a. true
myelofibrosis - features of blood cells
Heaps of platelets
teardrop shaped RBCs of varying sizes (anisocytosis)
symptoms that warrant immediate referral in children
unexplained petechiae
hepatosplenomegaly
symptoms that warrant urgent referral
repeat attendance at GP
with the same problem
no clear diagnosis
new neuro symptoms
abdominal mass
complications during cancer in children that are emergencies
raised ICP
sepsis/febrile neurtopenia
spinal cord compression
mediastinal mass
tumour lysis syndrome
presentation of sepsis in children
fever (or low temp)
rigors
drowsiness
shock (tachycardia, tachypnoea/hypotension)
prolonged capillary refill time
reduced UO
metabolic acidosis
presentation of raised ICP (early signs)
early morning headache and vomiting
tense fontanelle
increasing HC
late signs of raised ICP
constant headache
papilloedema
dilopia (VI palsy)
loss of upgaze
neck stiffness
status epilepticus
reduced GCS
cushings triad (low HR, high BP)
investigation for raised ICP
CT - screening
MRI - diagnosis
management of raised ICP
due to tumour - dexamethasone (Reduced oedema and increases CSF flow) ) IV STAT
or neurosurgery - urgent CSF diverversion (ventriculostomy) - hole in membrane at base of 3rd ventricle
EVD (temporary)
VP shunt
how does spinal cord compression occur in childhood cancer
invasion from paravertebral disease - 40% via intervertebral foramina (Extradural)
vetebral body compression
CSF seeding - intradural
direct invasion
presentation of spinal cord compression
weakness
pain
sensory
sphincter disturbance
management of spinal cord compression
urgent MRI
-> dexamethasone to reduce peri-tumour oedema
chemotheraphy - definite treatment when rapid response is expected
presentation of superior vena cava syndrome
risk due to lymphoma, neuroblastoma, tumours
facial , neck and upper thoracic oedema , plethora,
cyanosis
distended veins
ill/anxious/low GCS
treatment of superior vena cava syndrome
keep uprigh
what is tumour lysis syndrome
large numbers of cancer cells break down rapidly and release contents into the blood stream
(risk of happening after treatment - chemo/radiation)
release (potassium, phosphate, uric acid and nucleic acids in the blood stream)
- overwhelems the bodys ability to remove them
key metabolic disturbances in tumour lysis syndrome
hyperkalaemia (arrythmias)
hyperphosphataemia (kidney damage)
hyperuricemia (crystals to form in kidneys)
hypocalcacmia - muscle spasms, cramps, seizures, or heart problems
symptoms of tumour lysis syndrome
nausea and vomiting
fatigue
muscle cramps and fatigue
abnormal heart rhythms
seizures
decreased urine output
metabolic derganged in tumour lysis syndrome includes
high
- postassium
urate
phosphate
low
- calcium
acute renal failure due to urate load and phophaste deposition in tubules
treatment for tumour lysis syndrome
ECG monitoring
hyperhydrate
electrolytes (never potassium)
diuresis
urice acid lowering - allopurinol/urate oxidase uricozyme
how to treat hyperkalaemia in tumour lysis syndrome
Ca Resonium
salbutamol
insulin
lowering uric acid
allopurinol
rasburicase - urate oxidase uricozyme
side effects of radiotheraphy (LT)
fibrosis /scarring
second cancer
reduced fertility
what is a green stick fracture
the bone bends and partially breaks on one side but not all the way through (trying to bend twig - it bends and breaks on one side but not snapping completely)
why are green stick fractures common in kids?
their bones are more flexible and softer
typically occur in the forearm of leg
what classification system is used to grade growth plate injuries
salter-harris
- complete (with or without displacement)
- extends through the metaphysis (small chip fracture into middle of long bone)
- extends through the epiphysis (end of bone)
- physeal fracture plus epiphyseal and metaphyseal
- compression of growth plate (squashed into the long bone)
why is the size of children significant in impact injuries
large impact
-> smaller target (absorbs same force of impact)
why to infants lose heat easily
large surface area: volume ratio
heat loss significant
why is the skeleton of children more easily damaged?
soft, springy
***INCOMPLETELY CALCIFIED
*
deforms rather than breaks
poor at absorbing energy
less protection for organs
how does the connective tissue inside children differ
**less elastic tissue connective tissue
**
-> shearing and degloving
**Crowding of poorly protected vital organs
**
-> liver , spleen, bladder
children have less elastic connective tissue
a. true
b. false
a. true
more shearing and de-gloving
how do small children thermoregulate
Newborns & Infants: Heavily depend on brown fat for warmth, especially right after birth. It is most active during the first few months of life.
young children are pokilothermic what does this mean
A poikilothermic animal (also called ectothermic) is one whose body temperature varies with the environment because it does not regulate its internal temperature metabolically. Instead, these animals rely on external heat sources like the sun to warm up.
why are children more likely to become hypogylcaemic
little glycogen will be stored in the liver
+ hypothermia (etc)
develops quickly in children who are sick
Metaphyseal “bucket-handle” or corner fractures –
are sign of?
Highly specific for abuse, often from forceful shaking or pulling.
Transverse Fractures
Caused by direct blows, which are unusual in young children unless inflicted.
Common sites: humerus, femur, tibia.
when might spiral fractures be concerning?
non-mobile infants
Caused by torsional (twisting) forces.
when might Epiphyseal Separation Fractures occur
salter harris classification
Occur due to excessive pulling or traction forces.
femur fractures are suspicious because?
high force needed
Spiral or transverse femur fractures in infants should prompt investigation for abuse.
Spiral or transverse femur fractures in infants should prompt investigation for abuse.
a. true
b. false
a. true
red flags for non acidental trauma
Fractures inconsistent with the given history.
No history or vague explanation for significant injury.
Injuries in non-mobile infants (e.g., femur fractures in newborns).
Delayed medical presentation.
Associated injuries: rib fractures, skull fractures, bruises in unusual locations.
Metaphyseal (Classic Metaphyseal Lesions - CMLs) / “Bucket-Handle” or “Corner” Fractures
are highly suspcious for abuse
a. true
b. false
a. true
Highly specific for abuse.
Caused by forceful twisting, pulling, or shaking.
Common in the distal femur, proximal tibia, and proximal humerus.
when are spiral fractures worrying
Caused by torsional (twisting) forces.
Can occur accidentally in toddlers but are concerning in non-mobile infants.
what is SCIWORA
Spinal Cord Injury Without Radiographic Abnormality.
It refers to a spinal cord injury that occurs without visible fractures or dislocations on X-rays or CT scans.
It is more common in children due to the flexibility of their spine, which allows for significant movement without bony injury.
why does spinal cord injury without radiological abnormally occur in kids
It is more common in children due to the flexibility of their spine, which allows for significant movement without bony injury.
MRI needed
how does SCIWORA occur
Occurs mostly in children under 8 years old.
Results from hyperflexion, hyperextension, or distraction forces (e.g., motor vehicle accidents, falls, or non-accidental trauma).
non haemolytic febrile transfusion reaction occurs when
RBC transfusion
increase in body temperate of < 1.5c
pause and assess risk of espsis
give paracetamol PO/IV
restart transfusion at a slower rate /increase observations
Idarucizumab is a reversal agent for dabigatran
a. true
b. false
a.true
A history of Mycoplasma pneumonia followed by anaemia and jaundice with hepatosplenomegaly points towards the diagnosis of what kind of anaemia
normocytic
cold AIHA.
Autoimmune haemolytic anaemia is characterised by a positive direct antiglobulin test (Coombs’ test)
cause of AIHA
AIHA is idiopathic in most cases but can also occur secondary to drugs, lymphoproliferative disorder, or infections.
why does reticulocytosis occurs with AIHA.
Increased reticulocytes in AIHA are because of a normal bone marrow that responds to anaemia by increasing the synthesis of reticulocytes.
Autoimmune haemolytic anaemia (AIHA) may be divided in to ‘warm’ and ‘cold’ types meaning?
what temperature the antibodies best cause haemolysis. It is most commonly idiopathic but may be secondary to a lymphoproliferative disorder, infection or drugs.
general features of haemolytic anaemia
- anaemia
- reticulocytosis
- low haptoglobin
- raised lactate dehydrogenase (LDH) and indirect bilirubin
- blood film: spherocytes and reticulocytes
- specific features of autoimmune haemolytic anaemia
- positive direct antiglobulin test (Coombs’ test).
Hypersegmented neutrophils on the blood film indicate
megaloblastic anaemia (macrocytic)
The haematinics blood test detects levels of serum B12 and folate - a deficiency of which produces megaloblastic anaemia, therefore this is the best test to confirm the findings on the blood film.
Hb electrophoresis is used to detect
haemoglobinopathies such as sickle cell or thalassemia.
how do hypersegmented neutrophils indicate megaloblastic ‘anaemia’ ? ?
B12/Folate - Essential in DNA synthesis
Without you cannot synthesise new DNA
Fast growing cells, therefore, cannot undergo extra rounds of division to make smaller cells
Big RBC, Big Nucleus in WBC & Big Tongue
Hypercalcaemia, renal failure, high total protein =
myeloma
high calcium***
young children and people with Down Syndrome are at risk of wha leukamia
acute lymphoid leaukiamia ALL
Lambert-Eaton myasthenic syndrome is commonly associated with
SCLC
Thymomas are commonly associated with
myathensia gravis
Venous thromoboembolism - length of anticoagulation
for provoked and unprovoked
provoked (e.g. recent surgery): 3 months
unprovoked: 6 months
A peripheral blood smear shows increased mature and immature granulocytes, including myelocytes and metamyelocytes.
CML
Imatinib is a tyrosine kinase inhibitor specifically targeting
BCR-ABL fusion protein, which is commonly associated with CML. It is the mainstay treatment for CML, addressing the underlying pathophysiology by inhibiting the abnormal signalling pathways that drive the disease.
typical pattern of disseminated intravascular coagulation (DIC). (blood picture)
DIC typical blood picture:
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
This is the typical pattern of DIC, however, not all of these are necessary for a diagnosis. Even if only three of the above are positive, it is still likely that DIC.
common cause of DIC
SEPSIS
why do patients with thalassaemia need blood transfusions lifelong
maintain the patient’s haemoglobin while simultaneously suppressing enhanced erythropoiesis. Patient’s receiving life-long transfusions also require iron chelation therapy as iron-overload would otherwise occur.
Hydroxyurea and hydroxycarbamide are actually the same medication.
yes
used in the prophylactic management of sickle cell anemia to prevent painful episodes
used in the management of Von Willebrand’s disease
Desmopressin
It can be given intranasally and only needs to be taken when the patient is on her period. It can also be given intravenously or by subcutaneous injection, for example in anticipation of major surgery or post-trauma.
