SICKLE-CELL ANAEMIA Flashcards
What is sickle-cell anaemia?
An autosomal recessive disorder in which production of abnormal haemoglobin results in Vado-occlusive crises
Haemolytic Anaemia
What causes sickle-cell anaemia?
An amino acid substation on the gene coding for the beta chain on chromosome 11 leading to production of HbS rather than HbS
What symptoms sickle-cell anaemia?
Pain in skeleton, chest and abdomen triggered by dehydration, cold, infection or hypoxia
Frequent infection
Seated growth or puberty
Visual floaters
What are the signs of sickle-cell anaemia?
Pallor
Jaundice
Tachycardia
Dactylitis (swelling of back of hands and feet)
Leg ulcers
Which blood test would you do for sickle-cell anaemia?
Newborn screening heel prick test
FBC AND RETICULOCYTE COUNT: anaemia
DNA-BASED ASSAYS: presence of HbSS
What does a sickle-cell anaemia blood film show?
Uncleared RBCs, sickle-shaped cells and Howell-Jolly Bodies
What is the first-line treatment of sickle-cell anaemia?
Paracetamol/Ibuprofen
Hydrocarbamide (increase production of HbF)
L-Glutamine (reduces the frequency of pain crises
Crizanlizumab (reduces frequency of pain crises
How is a painful crises treated?
IV Analgesia
Treat Infection
Septic Screen
IV fluid
Keep warm and give oxygen
Blood transfusion
Penile Aspiration
What is Painful Crisis?
Sickle cells clog capillaries causing distal ischaemia
Associated with dehydration and haematocrit, pain, fever and symptoms of triggering infection
What is Splenic Sequestration Crisis?
Caused by RBCs blocking blood flow within spleen
Symptoms; acute and painful splenomegaly, severe anaemia, hypovolaemic shock
What is an aplastic crisis?
Temporary loss of the creation of new blood cells commonly triggered by parvovirus B19
Self-limiting within 2weeks
What is acute chest syndrome
Caused by infection or vasi-occlusion
Diagnosed by fever/respiratory symptoms + new infiltrates seen on CXR
Symptoms: pain, fever, tachypnoea, wheeze, cough
Treatment: bronchodilator, oxygen, analgesia, antibiotic, blood transfusion
