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Sickle Cell Anaemia
- commoner in Africans
- Abnormal haemoglobin (HbS) precipitates at low oxygen tensions
- this leads to sickle shaped red blood cells which that block small blood
vessels leading to painful crises
Clinical Features
- severe haemolytic
- coped with well because of oxygen dsissociation from HbS
- crises
- vaso-occlusive
- visceral
- aplastic
- haemolytic
Vaso-occlusive crisis
- precipitants
- infection
- acidosis
- dehydration
- deoxygenation
- ulcers of lower legs
- proliferative retionpathy
- priapism
- chronic liver damage
- pigment gallstones
- renal medullary infarcts (papillary necrosis)
- osteomyelitis
Visceral crisis
- sickling within organs leading to pooling of blood and exacerbation of
anaemia
- acute sickle chest syndrome
- dyspnoea
- falling PaO2
- chest pain
- pulmonary infiltrates
Aplastic crises
- parvovirus / folate deficiency
- reduction in reticulocyte levels
Investigation
- FBC
- Blood film
- haemoglobin electrophoresis
Treatment
- prophylactic
- folic acid 5mg
- good general nutrition and hygeine
- vaccination
- pneumococcu
- meningococcus
- haemophilus
Crises
- warmth
- IV fluid
- analgesia
- antibiotics
- exchange transfusion
- hydroxyurea
- stem cell transplantation
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