Paeds haem Flashcards
What can be used to increase the production of fetal haemoglobin in patients with sickle cell anaemia?
Hydroxycarbamide
What is the most common cause of anaemia in infancy?
Physiologic anaemia of infancy
When is there a normal dip in haemoglobin in healthy term babies? What is the cause?
- 6-9 weeks of age
- High Hb at birth -> high oxygen delivery to tissues -> negative feedback -> decreased erythropoietin production -> decreased Hb production
What are the causes of microcytic anaemia?
TAILS:
- Thalassaemia
- Anaemia of chronic disease
- Iron deficiency anaemia
- Lead poisoning
- Sideroblastic anaemia
What are the causes of normocyctic anaemia?
3 As and 2 Hs
- Acute blood loss
- Anaemia of chronic disease
- Aplastic anaemia
- Haemolytic anaemia
- Hypothyroidism
How can macrocytic anaemia be divided? Which type if vitamin deficiency caused by?
- Megaloblastic anaemia - B12 and folate deficiency
- Normoblastic macrocytic anaemia
What 2 symptoms are specific to iron deficiency anaemia?
- Pica
- Hair loss
What does a high level of reticulocytes in the blood indicate?
That anaemia is due to haemolysis or blood loss
What is the second most common type of leukaemia in children?
Acute myeloid leukaemia
What age group does ALL peak?
Ages 2-3
Leukaemia:
1) What presentation warrants referral for immediate specialist assessment?
2) If leukaemia is suspected what needs to be completed within 48 hrs
3) What would this show?
4) What are other investigations?
- Child with unexplained petechiae or hepatomegaly
- Full blood count
- Anaemia, leukopenia, thrombocytopenia, high number of abnormal white cells
- Blood film (blast cells), bone marrow biopsy
What is ITP characterised by?
Idiopathic (spontaneous) thrombocytopenia (low platelet count) causing a purpuric rash
What is ITP caused by?
A type II hypersensitivity reaction
What age group does ITP usually present?
Children under 10
What often precedes ITP?
Viral illness
What is the typical onset of symptoms in ITP?
24-48hrs
When is treatment required in ITP? What is the treatment?
- If the patient is actively bleeding/severe thrombocytopenia (plat < 10)
- Pred, IVIG, blood transfusions, platelet transfusions
What variant of haemoglobin do patients with sickle cell have? What does it cause?
- HbS
- HbS causes rbc to be an abnormal sickle shape
What is the inheritance pattern of sickle cell anaemia?
Autosomal recessive
When is sickle cell disease tested for?
On the newborn screening heel prick test at 5 days of age
What is sickle cell crisis? Give 4 examples
An umbrella term for a spectrum of acute crises related to the condition
- Vaso-occlusive crisis
- Splenic sequestration crisis
- Aplastic crisis
- Acute chest syndrome
What causes sickle cell crisis?
Can occur spontaneously or are triggered by dehydration, infection, stress, cold weather
What is the management of sickle cells crises?
- Supportive
- Low threshold for hospital addmission
- Treat infection that may have causes crisis
- Keep warm
- IV fluids
- Analgesia
What is the inheritance pattern of thalassaemia?
Autosomal recessive
What is the overall effect of thalassaemia?
Varying degrees of anaemia
Why can you get a pronounced forehead and molar eminences in thalassaemia?
Bone marrow expands to produce extra rbc to compensate for chronic anaemia
Why are patients with thalassaemia at risk of iron overload?
- Faulty creation of rbc
- Recurrent transfusions
- Increased absorption of iron in the gut in response to anaemia
What is the management of iron overload in thalassaemia?
- Limit transfusions
- Iron chelation
A patient presents with anaemia and jaundice following an infection with parvovirus. What is the underlying diagnosis?
Hereditary spherocytosis
Hereditary spherocytosis:
1) What happens?
2) What is the inheritance patterns?
3) How does it commonly present?
- Rbc are sphere shaped meaning they are fragile and easily destroyed when passing through the spleen
- AD
- Jaundice, anaemia, splenomegaly, gallstones
What is the inheritance pattern of G6PD deficiency?
X linked recessive
What can trigger G6PD deficiency crises?
- Infections
- Medications (antimalarials)
- Fava beans (broad beans)
How does G6PD present?
- Neonatal jaundice
- Anaemia
- Splenomegaly
What are seen on blood film in G6PD?
Heinz bodies
What the management of severe ITP
- Prednisolone
- IVIG
- Blood tranfusions
- Platelet transfusions (not long lasting)
What type of leukaemia is associated with Down’s syndrome?
AML
What cells are seen on lymph node biopsy in Hodgkins lymphoma?
Reed-Sternberg cells
What staging is used for Hodgkins lymphoma?
Ann Arbor
What happens in aplastic crisis (SCD)? What can it be triggered by?
- Temporary loss of creation of new blood cells
- Triggered by parvovirus B19 infection
