Sickle Cell Crisis Flashcards
Sickle cell anaemia is characterised by periods of good health with intervening crises.
Name the 4 types of crisis?
- Thrombotic
- Sequestration
- Aplastic
- Haemolytic
What crises is caused by an infection with parvovirus and has a sudden fall in haemoglobin?
Aplastic crises
Acute sickle chest syndrome
Tell me about the classic features of acute sickle chest syndrome & why it is important to know it?
It is the most common cause of death after childhood
- Dyspnoea
- chest pain
- pulmonary infiltrates
- ABG shows a low pO2
What crises is acute sickle cell chest syndrome seen in?
Sequestration Crises
What is also known as a painful crises or vaso-occlusive crises?
Thrombotic crises
What can precipitate a thombotic crises?
- Infection
- Dehydration
- Deoxygenation
What is the characteristic pathology of thrombotic crises?
-
Infarcts occur in various organs including:
- The bones (Avascular necrosis of hip)
- Hand-foot syndrome in children (redness, swelling, and pain on the palms of the hands and/or the soles of the feet.)
- Lungs
- Spleen
- Brain
What happens in a sequestrian crises?
Sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia.
What is the rarest crises and what happens in it?
A haemolytic crises
A fall in haemoglobin due an increased rate of haemolysis
A 22-year-old man with sickle cell anaemia presents with pallor, lethargy and a headache. Blood results are as follows:
- Hb - 4.6
- Reticulocyte count 3%
Infection with a parvovirus is suspected. What is the likely diagnosis?
- Thrombotic crisis
- Sequestration crisis
- Transformation to myelodysplasia
- Haemolytic crisis
- Aplastic crisis
Aplastic crisis
The sudden fall in haemoglobin without an appropriate reticulocytosis (3% is just above the normal range) is typical of an aplastic crisis, usually secondary to parvovirus infection
