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Medicine Notes Haematology Notes

Sickle Cell Anaemia Notes

Updated Sickle Cell Anaemia Notes

Haematology Notes

Haematology

Approximately 20 pages

Clinically relevant notes covering the main subjects within Haematology including malignancies, anaemias and pre-malignant disorders. Concise but with enough depth to answer SAQs for Finals. I have also included some notes on history and examination of a haematology patient - very useful for OSCEs.
These notes greatly aided me in passing my final exams with distinction. The notes were made using information from a variety of text books, lecture notes and workbooks. Each topic is colour-coded....

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The following is a more accessible plain text extract of the PDF sample above, taken from our Haematology Notes. Due to the challenges of extracting text from PDFs, it will have odd formatting:

Sickle Cell Anaemia

An inherited disorder of haemoglobin

Aetiology

  • Autosomal recessive - Point mutation (Glutamate to valine) on B haemoglobin chain

  • Causes hydrophobic pocket and sickling of cells

  • Genotypes

    • Homozygous Sickle Cell Anaemia

      • HbSS

      • Commonest and most severe

    • HbSC

      • One B chain sickled

      • One C chain

      • Similar to HbSS but more eye problems

    • Sickle cell trait

      • One normal and one affected B chain

Screening

  • Dependent on prevalence in region

  • High prevalence

    • Maternal blood test

    • If positive – father is tested

  • Low prevalence

    • Questionnaire

  • Newborn screening as part of the heel prick (Guthrie) test

Pathology

  • Sickled haemoglobin has low affinity for O2

  • Polymerises in low O2 tensions

  • Blocks microcirculation causing infarction

Clinical features

  • Severe haemolytic anaemia

  • Chronic condition punctuated by ‘crises’

Management

Chronic

  • Prophylactic 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)

    • Increased fetal Hb in blood = increased O2 carrying capacity

  • Transfusions – beware of Iron overload

  • Stem cell transplant – attempted cure

Acute - Vaso-occlusive crisis

  • Most common presentation to hospital

  • Sickling within bone marrow vasculature = pain

In the history

  • Is the pain like their normal sickle pain?

  • What home analgesia have they used?

  • Precipitants

  • Symptoms of infection

    • More susceptible as hyposplenic

    • Lower threshold for treating

Investigations

  • Bloods

    • FBC (Hb, WCC, reticulocyte count)

    • Group and save

    • U+E, LFT

    • Cultures if pyrexial

  • CXR

Management

  • O2

  • 1st dose of potent...

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