paediatric haematology Flashcards
what are causes of anaemia in infancy?
- physiological
- anaemia of prematurity
- blood loss
- haemolysis
- twin- twin transfusion (blood is unequally distributed)
what are common causes of haemolytic anaemia?
- Rh or ABO incompatability
- hereditary spherocytosis
- G6PD deficiency
why do you get physiological anaemia?
- get it at 6-9 weeks
- this is because getting higher levels of oxygen, which causes negative feedback
- production of erythropoetin is then surpressed
- then there is less production of haemoglobin by the bone marrow
what are the 3 types of anaemia?
Microcytic anaemia (low MCV indicating small RBCs)
Normocytic anaemia (normal MCV indicating normal sized RBCs)
Macrocytic anaemia (large MCV indicating large RBCs)
what are causes of microcytic anaemia?
T – Thalassaemia A – Anaemia of chronic disease I – Iron deficiency anaemia L – Lead poisoning S – Sideroblastic anaemia- body unable to put iron into haemoglobin
what are causes of normocytic anaemia?
A – Acute blood loss A – Anaemia of Chronic Disease A – Aplastic Anaemia H – Haemolytic Anaemia H – Hypothyroidism
what are causes of macrocytic anaemia?
- B12 and folate deficiency
what are the main reasons for iron deficiency anaemia in children?
Dietary insufficiency. This is the most common cause in children.
Loss of iron, for example in heavy menstruation
Inadequate iron absorption, for example in Crohn’s disease- absorbed by the duodenum and jejunum- requires acid so proton pump inhibitors can interfere with this process
what happens in ITP?
idiopathic (spontaneous) thrombocytopenia (low platelet count) causing a purpuric rash (non-blanching rash).
who is typically affected by ITP?
usually present in children under 10 years old. Often there is a history of a recent viral illness. The onset of symptoms occurs over 24 – 48 hours:
when should ITP be treated?
- depends on ow low the platelet count falls- ussually monitor until normal- 70% remit in 3 months
tx only if bleeding or severe thrombocytopenia (<10)
what are the treatment options for ITP?
Prednisolone
IV immunoglobulins
Blood transfusions if required
Platelet transfusions only work temporarily
what is some advice for ITP?
Avoid contact sports
Avoid intramuscular injections and procedures such as lumbar punctures
Avoid NSAIDs, aspirin and blood thinning medications
Advice on managing nosebleeds
Seek help after any injury that may cause internal bleeding, for example car accidents or head injuries
what are complications of ITP?
Chronic ITP
Anaemia
Intracranial and subarachnoid haemorrhage
Gastrointestinal bleeding
which gene is affected in sickle cell?
beta globin on ch11
which infection can cause aplastic crisis in sickle cell patients?
Aplastic crisis describes a situation where there is temporary loss of the creation of new blood cells. This is most commonly triggered by infection with parvovirus B19.
when is sickle cell checked for?
heal prick test at age 5 days
what is the treatment for sickle cell crisis?
- supportive management
tx infection, keep warm, IV fluids
what happens in sickle cell vaso-oclusive crisis?
- painful crisis
sickle shaped blood cells clogging capillaries and causing distal ischaemia. It is associated with dehydration and raised haematocrit. Symptoms are typically pain, fever and those of the triggering infection
what is splenic sequestration crisis?
in sickle cell blood flow is blocke din the spleen leasing to enlarged spleen, severe anaemia nand circulatory collapse-> splenectomy can prevent this
what to do for acute chest syndrome in sickle cell patients?
this is a medical emergency with high mortality
Antibiotics or antivirals for infections
Blood transfusions for anaemia
Incentive spirometry using a machine that encourages effective and deep breathing
Artificial ventilation with NIV or intubation may be required
what is the inheritence pattern of hereditary spherocytosis?
dominant- founs in northern europeans
what happens in hereditary spherocytosis?
RBC sphere shaped and break up as theu go through the spleen
- parvovirus infection can lead to an aplastic crisis in these patients
what is the presentation for heriditary spherocytosis?
Jaundice
Anaemia
Gallstones
Splenomegaly
how is hereditary spherocytosis diagnosed?
- spherocytes in blood film
- mean corpuscular HB conc raised
- raised reticulocytes (due to rapid cell turnover)
how is hereditary spherocytosis managed?
folate supplementation and splenectomy.
Removal of the gallbladder (cholecystectomy) may be required if gallstones are a problem.
Transfusions may be required during acute crises.
what is the pattern of inheritence for G6PD deficiency?
- X linked recessive
how does G6PD deficiency make someone more susceptible to haemolysis?
A deficiency in G6PD makes cells more vulnerable to ROS, leading to haemolysis in red blood cells. Periods of increased stress, with a higher production of ROS, can lead to acute haemolytic anaemia.
what can be seen in blood fillm with G6PD deficiency?
Heinz bodies may be seen on a on blood film. Heinz bodies are blobs of denatured haemoglobin (“inclusions”) seen within the red blood cells.
Diagnosis can be made by doing a G6PD enzyme assay.
what is managedmnt of GPD deficiency?
avoid triggers like fava beans and certain medications
what can cure alpha thalaseamia?
bone marrow transplant
