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Sickle Cell Anaemia Notes

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This is an extract of our Sickle Cell Anaemia document, which we sell as part of our Haematology Notes collection written by the top tier of University Of Leicester students.

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Sickle Cell Anaemia An inherited disorder of haemoglobin

AetiologyAutosomal recessive - Point mutation (Glutamate to valine) on B haemoglobin chain Causes hydrophobic pocket and sickling of cells Genotypes o Homozygous Sickle Cell Anaemia
? HbSS
? Commonest and most severe o HbSC
? One B chain sickled
? One C chain
? Similar to HbSS but more eye problems o Sickle cell trait
? One normal and one affected B chain

ScreeningDependent on prevalence in region High prevalence o Maternal blood test o If positive - father is tested Low prevalence o Questionnaire Newborn screening as part of the heel prick (Guthrie) test

PathologySickled haemoglobin has low affinity for O2 Polymerises in low O2 tensions Blocks microcirculation causing infarction

Clinical featuresSevere haemolytic anaemia Chronic condition punctuated by 'crises'

Triggers for a crisis
- Infection
- Dehydration
- Change in temp.
- Stress

Management ChronicProphylactic advice (avoid triggers) Folic acid 5mg OD Prophylactic abx. (splenic autoinfarction = 'asplenic' - Lifelong penicillin V) Vaccinations (against encapsulated bacteria - hyposplenic) Hydroxycarbamide - increases HbF levels (give if >3 crises in 12 months) o Increased fetal Hb in blood = increased O2 carrying capacity Transfusions - beware of Iron overload Stem cell transplant - attempted cure

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