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on 05-Aug-2015 (Wed)

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Flashcard 150902779

Tags
#haematology #medicine
Question
What is the pathophysiology of Sickle-cell anaemia ?
Answer
  • Production of abnormal globin chains.
  • An amino acid substitution in the gene coding for the chain (Glu Val at position 6 ) results in the production of HbS rather than HbA.
  • HbS polymerizes when deoxygenated, causing RBCs to deform, producing sickle cells, which are fragile and haemolyse, and also block small vessels
  • ​HbA 2 and HbF are still produced.


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Flashcard 150902789

Tags
#haematology #medicine
Question
What virus causes the Aplastic crisis with sickle cell anaemia ?
Answer
parvovirus B19


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Flashcard 150902796

Tags
#haematology #medicine
Question
Who does a Sequestration crisis of sickle cell anaemia effect ?
Answer
Children as the spleen has not yet undergone atrophy


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Flashcard 150902803

Tags
#haematology #medicine
Question
What occurs in a Sequestration crisis of sickle cell anaemia ?
Answer
There is pooling of blood in the spleen ± liver, with organomegaly, severe anaemia and shock. Urgent transfusion is needed


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Flashcard 150902810

Tags
#haematology #medicine
Question
What are the possible complications of Vaso-occlusive ‘painful’ crisis in sickle cell anaemia ?
Answer
  • Dactylitis
  • Mesenteric Ischaemia
  • Strokes and Seizures
  • Avascular Necrosis
  • Priapism ​​​​


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Flashcard 150902817

Tags
#haematology #medicine
Question
What is the pathphysiology behind Vaso-occlusive ‘painful’ crisis in sickle cell anaemia ?
Answer
Microvascular occlusion


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Flashcard 150902824

Tags
#haematology #medicine
Question
How long does the Aplastic crisis with sickle cell anaemia last ?
Answer
Usually self-limiting <2 wks


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Flashcard 150902831

Tags
#haematology #medicine
Question
What are the Complications of sickle cell anaemia ?
Answer
  • Splenic infarction ( <2 yrs old, due to micro vascular occlusion, leading to susceptibility to infection)
  • Poor growth
  • Chronic renal failure
  • Gallstones
  • Retinal disease
  • Iron overload or blood-borne infection after repeated transfusion
  • Lung damage: Hypoxia, Fibrosis, Pulmonary Hypertension


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