Hereditary Spherocytosis and Haemolytic Anaemia intro Flashcards
1
Q
Inherited causes
A
Hereditary Spherocytosis Hereditary Elliptocytosis Thalassaemia Sickle Cell Anaemia G6PD Deficiency
2
Q
Acquired causes
A
Autoimmune haemolytic anaemia Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn) Paroxysmal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve related haemolysis
3
Q
Hereditary Spherocytosis
What is it
Clinical Features
A
Autosomal dominant defect
Results in sphere shapedred blood cellsthat easily break down when passing through the spleen
CLINICAL FEATURES Jaundice Gallstones Splenomegaly Aplastic crisis precipitated by parvovirus infection
4
Q
Hereditary Spherocytosis
Investigations
A
FBC: Normocytic Anaemia, high reticulocytes
Blood film: spherocytes
Osmotic fragility test was previously recommend choice, not anymore
EMA binding
Coombs test to rule out autoimmune haemolytic anaemias
5
Q
Hereditary Spherocytosis
Management
A
Splenectomy
Oral folate supplements
