Paediatric Haematology Flashcards
What is kernicterus?
This is when free bilirubin crosses the blood-brain barrier and deposits in the basal ganglia
A child presents with sudden onset of purpura and petechiae and the child is otherwise well despite having had a viral illness a few weeks ago. What is the most likely diagnosis?
Idiopathic Thrombocytic purpura
What will be seen on a blood count in ITP
Low platelet count with normal white and red blood cells
What must be ruled out in ITP?
Leukaemia
Take a bone marrow aspirate prior to starting any treatments for ITP
What is the most common cause of haemolysis in children?
Hereditary spherocytosis
What mode of inheritance is hereditary spherocytosis?
Autosomal Dominant
What viral infection can cause transient severe anaemia?
Parvovirus B19
What is the most common acquired bleeding disorder in children?
Idiopathic Thrombocytic Purpura
What is Henoch-Schonlein Purpura?
An acute immune complex mediated vasculitis.
What is the common triad of symptoms in HSP?
Purpura
Arthritis / arthralgia
Abdominal pain
Explain the physiology of rhesus haemolytic disease:
A rhesus -ve mother will deliver a rhesus +ve baby. The leak of foetal blood into the mothers circulation will cause her to mount an immune response of anti-D IgG antibodies.
This will then affect any subsequent pregnancies with rhesus +ve babies.
What is the clinical consequence of rhesus disease?
The IgG antibodies will cause haemolysis of the red blood cells in the foetus, causing anaemia. This will then lead to the development of hydrops fetalis
What is hydrops fetalis?
This is the abnormal accumulation of fluid in two or more compartments e.g. ascites, pericardial effusions etc which carries a high rate of mortality.
How is rhesus haemolytic disease prevented?
All pregnant women who are rhesus -ve are given a rhesus D antibody vaccine at 28 weeks gestation
When is newborn jaundice concerning?
Within the first 24 hours or after 2 weeks
An otherwise apparently healthy 3 week old newborn presents with prolonged jaundice, clay coloured stools and very yellow urine. Parents are concerned because they can feel a “mass” in the babies tummy. What is the likely diagnosis?
Biliary atresia
The parents can feel the spleen / liver
What is the treatment for biliary atresia?
If it is caught within the first 6 weeks of life the Kasai procedure is done to improve biliary drainage. Past 6 weeks the child will be in acute liver failure and will require a transplant.
What is the Kasai procedure?
A hepatoportoenterostomy
This is where the extrahepatic biliary tree is identified and an intestinal limb is attached to the porta hepatis to allow for bile drainage.
What is the pathology of biliary atresia?
There is an absence of intra- or extrahepatic bile ducts which leads to a conjugated hyperbilirubinemia causing obstructive jaundice. If untreated it will lead to liver failure and become fatal.
Mild physiological jaundice is common in premature newborns, true or false?
True
Due to liver immaturity
What group of (term) babies are more prone to developing jaundice?
Breastfed babies
What should be measured to exclude conjugated hyperbilirubinemia in suspected breastfed jaundice?
Split bilirubin
What chronic illness can lead to cholestasis in the newborn?
cystic fibrosis
How is biliary atresia diagnosed?
Rising conjugated bilirubin levels
Diagnostic isotope scan
A deficiency of _______ can lead to hemorrhagic disease of the newborn, causing abnormal bruising and bleeding?
Vitamin K
What is the most common childhood malignancy?
Leukaemia
What is the peak age for acute lymphoblastic leukaemia?
2-5 years old
What are some of the symptoms of ALL?
Symptoms of pancytopenia - pallor, infection and bleeding
Fatigue
Anorexia
Malaise
Lymphadenopathy / painless lumps in armpit, neck and groin
What is tumour lysis syndrome?
This is when the breakdown of large numbers of malignant cells leads to very high serum urate, phosphate and potassium levels.
Urate crystals can cause kidney failure,
How should febrile neutropenia be managed?
Significant fever or signs of infection in a neutropenic patient should be investigated thoroughly and treated with aggressive broad spectrum antibiotic therapy.
What is the best way to prevent tumour lysis syndrome?
Good hydration
use of allopurinol
What should immunosuppressed children not be given as they undergo treatment for leukaemia (or other cancer) and what should be given if they have contact with varicella virus?
Live vaccines
Give a specific immunoglobulin. If they develop varicella treat with aciclovir
What is the 5 year survival rate for ALL in children who have appropriate treatments?
80-95%
