Paediatric Haematology Flashcards
Why does physiological anaemia of infancy occur?
High haemoglobin levels at birth mean high oxygen delivery to the tissues at birth. This has a negative feedback effect on EPO so there is reduced production of haemoglobin by the bone marrow
What are some reasons that cause premature neonates to becomes anaemic?
Less time in utero receiving iron from the mother
RBC production cant keep up with rapid growth in first few weeks
Reduced EPO levels
Blood tests remove from their circulating volume
What are the two most common causes of anaemia in older children?
Iron deficiency and blood loss
What are causes of microcytic anaemia? (TAILS)
Thalassaemia, anaemia of chronic disease, iron deficiency, lead poisoning, sideroblastic anaemia
What are causes of normocytic anaemia? (3As and 2Hs)
Acute blood loss, aplastic anaemia, anaemia of chronic disease
haemolysis, hypothyroidism
What are causes of macrocyctic anaemia?
B12 and folate deficiencies, hypothyroidism, alcohol, reticulocytosis,
What are symptoms of anaemia?
Tiredness, palpitations, breathlessness, headaches, dizziness
What are some specific signs that would indicate iron deficiency anaemia?
Pica, hair loss/brittle hair and nails, koilonychia, angular chelitis, atrophic glossitis
Jaundice occurs with which cause of anaemia?
Haemolytic anaemia
Bone deformities are more associated with which cause of microcytic anaemia?
Thalassaemia
From most to least common list the types of leukaemia seen in children
Acute lymphoid leukaemia
Acute myeloid leukaemia
Chronic myeloid leukaemia
What ages are ALL and AML most commonly seen?
ALL common between 2-5 but peaks between 2-3
AML peaks under 2 years of age
What is the pathophysiology of leukaemia and why does this lead to pancytopenia?
A genetic mutation in a precursor cell in the bone marrow lead to excessive production of a single type of abnormal white blood cell.
This leads to suppression of the other cell lines leading to pancytopenia
What is pancytopenia a combination of?
Anaemia
Leukopenia
Thrombocytopenia
What is the main environmental risk factor for leukaemia?
Radiation in utero e.g from abdominal X-ray of mum while pregnant
What are some other conditions which can predispose someone to a higher risk of developing leukaemia?
Downs, kleinfelter syndrome, fanconi’s anaemia, noonan syndrome
Although the presentation of leukaemia can be quite non-specific, what are some symptoms?
Leukopenia: recurrent infections
Thrombocytopenia: bleeding and abnormal bruising
Anaemia: fatigue, pallor
Unexplained fever, failure to thrive, weight loss, night sweats, bone pain, hepatosplenomegaly, generalised lymphadenopathy
If leukaemia is suspected then what investigation would be requested and in what timeframe?
Full blood count within 48 hours
What investigations can be done to establish the diagnosis of leukaemia?
Full blood count (anaemia, leukopenia, thrombocytopenia)
Blood film (blast cells)
Bone marrow biopsy
Lymph node biopsy
What further tests can be done to stage the leukaemia?
Chest X ray
CT scan
Lumbar puncture
Genetic analysis of abnormal cells
What is sickle cells disease?
A genetic condition that causes sickle shaped red blood cells which are more likely to stick together and be easily destroyed
What is the inheritance pattern of sickle cell disease?
Autosomal recessive
What is the relationship with sickle cell disease and malaria?
Sickle cell is more common in people from areas traditionally affected by malaria and having one copy of the gene (sickle cell trait) reduces the severity of malaria
When are babies first screened for sickles cell disease?
Newborn blood spot test on day 5
What are some complications of sickles cell disease?
Anaemia, acute chest syndrome, sickle cell crisis, chronic kidney disease, increased risk of infection, stroke, avascular necrosis
What is priapism?
Persistent and painful erection occurring when blood gets trapped in the penis- urological emergency treated by aspirating blood from penis
Although sickle cell crises can occur spontaneously, what are some triggers?
Dehydration, infection, cold weather or stress
How are sickle cell crises managed?
Analgesia, fluids, keep warm
Admit to hospital
Treat any underlying infections
Infection with which virus usually triggers an aplastic crisis?
Parvovirus B19
How will acute chest syndrome present and what will be seen on chest x ray?
Fever, dyspnoea, chest pain, cough and hypoxia
Pulmonary infiltrates
what are the general principles of management for sickle cell disease?
Avoid triggers
Stay up to date with vaccinations
Prophylactic ABx
Hydroxycarbamide
Crizanlizumab
Blood transfusions for severe anaemia
Bone marrow transplant (can be curative)
Why do we give hydroxycarbamide in sickle cell disease?
Promotes production of fetal Hb which does not lead to sickling of RBCs.
It reduces vaso-occlusive crises and anaemia
Why do we use crizanlizumab for sickle cell disease?
This monoclonal antibody targets p-selectin which is an adhesion molecule on endothelial cells and platelets.
Prevents RBCs sticking to blood vessel walls and reduces vaso-occlusive crises
What is the likely underlying diagnosis for a patient who develops anaemia and becomes jaundice after eating broad beans/developing an infection or being treated with anti-malarials?
G6PD deficiency
Is lymphoma more commonly seen in younger or older children?
Older children
