Anaemias Flashcards
What is Aplastic Anaemia?
A pancytopenia due to destruction of the HSC
Name three causes of Aplastic Anaemia
Genetic - Fanconi Anaemia
Radiation
Infection - HIV/EBV
Name two other features of Fanconi Anaemia
Predisposition to malignancy
Short Stature
Name two investigations for Aplastic Anaemia
Bloods (inc EPO)
BM Biopsy (dry tap)
How is Aplastic Anaemia managed?
<50 - SCT
>50 - Immunosupression +/- GCSF +/- Transfusions
What characterises autoimmune haemolytic anaemia?
Anaemia
Increased Reticulocytes
Reduced Haptoglobin
+ve Coombs
Sphero and Reticulocytes
What is Warm AHA?
IgG causes haemolysis at extravascular sites at body temperature
Name two associations of Warm AHA
CLL
SLE
What is Cold AHA?
IgM causes haemolysis at 4 degrees intravascularly generally causing raynaud symptoms alongside typical haemolytic anaemia picture
How is AHA managed?
Steroids +/- Rituximab
What is Beta Thalassaemia major?
Comlete absence of beta globulin chains presenting in first year of life with failure to thrive
How is Beta Thalassaemia physiologically compensated?
Increase in HBA2 and HBF
How is Beta Thalassaemia Major managed?
Repeated Transfusion
Desferrioxamine
How does Beta Thalassaemia trait present?
Typically asymptomatic as only reduced production
Name three hereditary haemolytic anaemias
Sickle Cell
G6PDH
Hereditary Spherocytosis
Name two immune haemolytic anaemias
AHA
Transfusion related
Name two non immune haemolytic anaemias
DIC
TTP/HUS
What is Hereditary Spherocytosis?
Most common hereditary HA
Autosomal abnormality in cytoskeleton - then destroyed by spleen
Name three features of H.Spherocytosis
Failure to thrive
Jaundice
Splenomegaly
How is H.Spherocytosis diagnosed?
Family History +Suggestive bloods + Symptoms