Paediatric Haematology Flashcards
What is Idiopathic Thrombocytopaenic Purpura?
Idiopathic Thrombocytopenic Purpura
Most common cause of thrombocytopenia in children
What is the cause of Idiopathic Thrombocytopaenic Purpura?
Autoimmune disorder causing destruction of platelets leading to reduced circulating platelet numbers
What is common in the history of children with Idiopathic Thrombocytopaenic Purpura?
Recent viral infection or immunisation
What age is Idiopathic Thrombocytopaenic Purpura most common?
2-10 yo
How does Idiopathic Thrombocytopaenic Purpura present?
Commonly asymptomatic
Can see: Purpura and superficial bleeding Petechiae Nosebleeds Intracranial haemorrhage Menorrhagia GI bleeds
Some have enlarged spleens
What differentials would you consider for Idiopathic Thrombocytopaenic Purpura?
Aplastic anaemia Leukaemia Von-Willebrand Meningococcal septicaemia HSP Non Accidental Injury
What investigations would you request if you suspect Idiopathic Thrombocytopaenic Purpura?
FBC
Peripheral blood smear
Can do Bone Marrow Aspirate
What would an FBC show in Idiopathic Thrombocytopaenic Purpura?
Thrombocytopenia but no pancytopenia
Characteristic increase in megakaryocytes
How does Idiopathic Thrombocytopaenic Purpura normally progress?
Normally an acute, benign self limiting condition
What is Idiopathic Thrombocytopaenic Purpura treatment based on?
Symptoms
NOT PLATELET COUNT
What is the general management for Idiopathic Thrombocytopaenic Purpura?
No contact sports
Avoid NSAID’s
Look out for red flags
When is pharmacological treatment suggested in Idiopathic Thrombocytopaenic Purpura?
Life-threatening bleeding
Increased risk of bleeding - haemophilia
Prior invasive procedure
What pharmacological management may be advised for Idiopathic Thrombocytopaenic Purpura?
1 Prednisolone
2 IV Immunoglobulin
3 Rituximab
4 High dose dexamethasone
What is the prognosis for Idiopathic Thrombocytopaenic Purpura?
80% completely recover within 6 months
Some children have chronic thrombocytopenia - higher risk in teenage girls
What is used for Idiopathic Thrombocytopaenic Purpura if medical therapies fail?
Splenectomy
What Hb level signifies anaemia in a child <5yo?
<11g/dl
What Hb level signifies anaemia in a child between 5-11yo?
<11.5g/dl
What Hb level signifies anaemia in a child between 12-14yo?
<12g/dl
How can anaemia be classified?
Colour - hypochromic/normochromic
Size - microcytic, normocytic, macrocytic
What symptoms would anaemia’s present with?
Fail to thrive Fatigue Short of breath Cardiac changes Growth affected but head circumference preserved Pallor Petechiae or bruising Splenomegaly - haemolysis? Jaundice Small stature, small head and frontal bossing
What cardiac changes could be seen in anaemia?
Exertional tachycardia
Gallop rhythm
Systolic flow murmur
What is the most common cause of anaemia in children?
Iron deficiency anaemia
What mechanisms can cause anaemia?
Decreased RBC or Hb production
Increased destruction
Blood loss
What are the causes of microcytic anaemia?
Defects in haem or globin synthesis:
Iron deficiency
Chronic inflammation
Thalassaemia
What are the causes of normocytic anaemia?
Haemolytic anaemias
Haemorrhage
Hypo-production
What causes macrocytic anaemia?
Bone marrow megaloblastic:
Vitamin B12 deficiency
Folic acid deficiency
Bone marrow not megaloblastic:
Hypothyroidism
Fanconi anaemia
What is fanconi’s anaemia?
Congenital aplastic anaemia - decreased bone marrow production of all blood cells
Give some causes of haemolytic anaemias
Red cell defects - spherocytosis, sickle cell, G6PD deficinecy
Extrinsic disorders - autoimmune mediated, hypersplenism, septic, DIC, haemolytic disease of the newborn
What could cause B12 deficiency?
Breast fed by vegetarian and in malabsorption
What could cause folate deficiency?
IBD
Coeliac
Anticonvulsants
What are some other causes for decreased RBC or Hb production?
Marrow infiltration with malignant cells
Marrow replaced with fibrous tissue in TORCH infections
What investigations would you request to investigate anaemia?
FBC and MCV B12 and folate Ferritin - iron deficiency Reticulocyte count Blood film Red cell enzyme studies Coomb's test - autoimmune haemolysis Endomysial antibodies - coeliac's
Why are pre-term infants at risk of Iron Deficiency anaemia?
Fetus absorb iron from mother across placenta
Term infants have adequate reserves for 4 months
Preterm run out by 8 weeks
Why is type of milk important when considering iron deficiency anaemia?
50% of iron absorbed from breast milk compared to 10% from cow’s
Formula milk has more iron but only 4% absorbed
What can affect iron absorption in the diet?
Enhanced by vit C
Reduced by tannin (in tea)
When does iron requirement increase for children?
Adolescence - esp girls who lose iron through menstruation
Give some causes of iron deficiency
Menstruation Hookwork infestation Repeated venesection in babies Meckel's diverticulum Recurrent epistaxis
How is iron deficiency managed?
Avoid unmodified cow’s milk
Iron supplements - sodium feredetate
In severe cases, may need transfusion
