Paediatric Haematology Flashcards
What is the pathogenesis of Anaemia?
1) Blood loss: Acute haemorrhage/chronic gut bleeding
2) Decreased Production: Nutritional deficiency, bone marrow failure, infiltration
3) Increased consumption: Acquired/Inherited
What are the main blood parameters?
1) HB (Normal for age range and sex)
2) Abnormality in single cell/multiple cell lines
3) MCV (Microcytic, normocytic, macrocytic)
What are some examples of MCV causes?
Micro: Iron def, Thalassaemia, sideroblastic
Normal: Acute blood loss, infection, renal failure, bone marrow infiltration
Macro: Aplastic anaemia, newborn
What do different reticulocyte levels indicate?
Low: Production problem e.g. Infection, renal disease, drugs, marrow failure/infiltration
High: Degradation problem e.g. bleeding/haemolysis
What is the most common cause of childhood anaemia?
Iron deficiency
What are the main signs of anaemia?
1) Pallor
2) Irritable
3) SOB
4) Lethargy
5) Tachycardic
What is treatment for IDA?
Ferrous Sulphate Tablets/Oral Iron
- Fails due to non-compliance
What causes increased destruction of RBC?
IntraCorpuscular: Hb, Enzyme or Membrane
ExtraCorpuscular: Autoimmune, fragmentation, hypersplenism and plasma factors
What are the main presentations of G6PD?
1) Neonatal jaundice
2) Chronic non-spherocytic haemolytic anaemia
3) Intermittent episodes of intravascular haemolysis
(X Linked recessive)
What are two types of Haemolysis?
1) Intravascular Haemolysis: Haemoglobinuria, rigors, fever, back pain
2) Sporadic Haemolysis: Drugs, fava beans, fever, acidosis
TREAT: Stop precipitant, transfusion and renal support
What is the inheritance pattern of SCD?
Autosomal recessive
What is the affect of sickle cell disease on Hb and reticulocyte count?
Low Hb.
Raised reticulocyte count.
What is the treatment for SCD?
1) Hydroxycarbamide
2) Transfusions
3) Stem cell transplants
What are the main problems of SCD?
Anaemia.
Infection.
Painful crises.
Stroke.
Acute chest infection/infarction.
Splenic sequestration
Spherocytosis: Cause, pattern and treatment?
C: Parovirus
P: AD
T: Splenectomy (^ RBC survival)
Name 2 coagulopathies
1) Von Willebrand Disease
2) Haemophilia
Describe VWD PP
Bleeding disorder due to an abnormality of vWF. vWF acts as an adhesive bridge between platelets and the damaged sub-endothelium. Without it it takes longer for bleeding to stop.
How is VWD inherited, presented and treated
In: AD/AR
P: Easy bruising, epistasis
Diagnosis: Clotting screen showing prolonged APTT (Affects factor 8!)#
Treat: No cure, instead manage with: Tranexamic acid, desmopressin, nebulised
Haemophilia: Pattern, Clotting factor, Presentation
Pattern: X Linked Recessive
Type A: Factor 8 (VIII) deficiency
Type B: Factor 9 deficiency
Presentations: Easy bruising, Haematoma, mouth/joint bleeds
What are 2 causes of Thrombocytopenia?
1) Marrow failure
2) ITP
What are 3 signs of Thrombocytopenia?
1) Petechial rash
2) Bruising
3) Bleeding
What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?
ITP –> Low platelets, normal blood film and clot
What can trigger acute ITP?
Viral infection
Give 3 signs of beta thalassaemia major.
Severe anaemia.
Jaundice.
Splenomegaly.
Failure to thrive.
