Haemolytic anaemia Flashcards
name 5 inherited haemolytic anaemia
- Hereditary Spherocytosis
- Hereditary Elliptocytosis
- Thalassaemia
- G6PD deficiency
- Sickle cell anaemia
3 features of haemolytic anaemia
- anaemia
- splenomegaly (due to spleen filling with destroyed blood cells)
- jaundice
blood film result in haemolytic anaemia
schistocytes
direct Coombs test in autoimmune haemolytic anaemia?
positive
Hereditary Spherocytosis inheritance?
autosomal dominant
shape of red blood cell in hereditary spherocytosis (from what to what)
biconcave disc to sphere shaped
what can precipitate aplastic crisis?
Parovirus infection
presentation of Hereditary Spherocytosis? (4)
- jaundice
- splenomegaly
- gall stones
- aplastic crisis
MCHC & reticulocytes in Hereditary Spherocytosis?
high MCHC high reticulocytes (rapid turnover of cells)
management of Hereditary Spherocytosis
- folate supplementation
- splenectomy
inheritance of G6PD Deficiency?
x linked recessive
triggers for G6PD Deficiency crises? (3)
- infections
- fava beans
- medications
what is seen on blood film with G6PD Deficiency?
Heinz bodies
presentation of G6PD Deficiency?
- neonatal jaundice
- gall stones
- anaemia
- splenomegaly
medications that trigger G6PD Deficiency related haemolysis?
- primaquine
- ciprofloxacin
- sulfonyureas
- sulfasalazine
2 types of autoimmune haemolytic anaemia? and why
warm & cold
depending on the temperature at which the autoantibodies that attack the red blood cells function at
causes of Warm Type Autoimmune Haemolytic Anaemia
idiopathic
what is cold autoimmune haemolytic anaemia also called?
cold agglutinin disease
what is cold AIHA often secondary to?
- Lymphoma
- Leukaemia
- lupus
- infections: CMV, EBV, HIV, mycoplasma
management of AIHA
- blood transfusions
- steroids
- rituximab
- splenectomy
Rituximab mechanism
Monoclonal antibody against B cells
Inheritance of sick cell anaemia?
Autosomal recessive
when is fetal haemoglobin (HbF) usually replaced by Haemoglobin A
6 weeks of life
abnormal gene in sickle cell anaemia?
gene for beta globin, on chromosome 11
what is the relation between sickle cell anaemia & malaria
having one copy of the gene/sickle cell trait, makes you more likely to survive malaria = selective advantage in malaria prevalent areas (e.g. Africa, India, Middle east, carribean)
triggers of vaso-occlusive sickle crisis? (4)
- cold
- dehydration
- infection
- hypoxia
general management of Sickle cell anaemia
- maintain hydration & warmth
- phenoxymethypenicillin
- Hydroxycarbamide
- blood transfusion
- bone marrow transplant
how to prevent infections in sickle cell patients?
Penicillin V/Phenoxymethylpenicillin OD
Management of sickle cell crises?
- admit
- treat infection
- keep warm & maintain hydration
- analgesia
- priapism
role of priapism in sick cell crises?
in a crises, the sickle blood cells clump together and block/affect the blood flow in penis… to manage this; ‘priapism’ - blood aspiration
what is a vaso-occlusive crisis?
sickle shaped blood cells clog up capillaries causing reduced blood perfusion distal to the blockage –> ischaemia
what happens in a splenic sequestration crisis?
red blood cells clump together and block blood flow within spleen. The pooling of blood in the spleen can lead to a severe anaemia and circulatory collapse.
what is aplastic crisis & what triggers it?
- temporary loss of creation of new RBC
- triggered by Parovirus B19 infection
what does Parovirus B19 infection trigger
aplastic crisis
diagnostic critera for acute chest syndrome (2)
- fever/ resp symptoms
- new infiltrates seen on x-ray
management of acute chest syndrome (4)
1) infection management: abx/antivirals
2) blood transfusion
3) NIV
4) incentive spirometry
