Sickle cell anemia Flashcards
(37 cards)
1
Q
Sickle-cell anaemia is an
A
autosomal recessive condition that results for synthesis of an abnormal haemoglobin chain termed HbS
2
Q
normal haemoglobin
A
HbAA
3
Q
sickle cell trait
A
HbAS
4
Q
homozygous sickle cell disease
A
HbSS
5
Q
Some patients inherit one HbS and another abnormal haemoglobin (HbC) resulting in
A
HbSC
6
Q
x
A
7
Q
x
A
8
Q
x
A
9
Q
x
A
10
Q
x
A
11
Q
x
A
12
Q
x
A
13
Q
x
A
14
Q
x
A
15
Q
x
A
16
Q
x
A
17
Q
x
A
18
Q
x
A
19
Q
x
A
20
Q
x
A
21
Q
x
A
22
Q
Sickle cell anaemia is characterised by periods of
A
good health with intervening crises
23
Q
types of crises are recognised?
A
thrombotic, ‘vaso-occlusive’, ‘painful crises’
acute chest syndrome
anaemic:
aplastic
sequestration
infection
24
Q
Thrombotic crises/ ‘vaso-occlusive’, ‘painful crises’ precipitated by ?
A
infection, dehydration, deoxygenation (e.g. high altitude)
25
Thrombotic crises/ 'vaso-occlusive', 'painful crises' diagnosed ?
clinically
26
infarcts occur in various organs including ?
bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
27
Acute chest syndrome?
vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma
28
cxr in acute chest syndrome ?
pulmonary infiltrates on chest x-ray
29
chest syndrome Mx
pain relief
respiratory support e.g. oxygen therapy
antibiotics: infection may precipitate acute chest syndrome and the clinical findings (respiratory symptoms with pulmonary infiltrates) can be difficult to distinguish from pneumonia
transfusion: improves oxygenation
30
Aplastic crises caused by
parvovirus
31
Diagnosis of aplastic crisis ?
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count
32
Sequestration crises clinical features ?
splenomegaly
33
Monitoring of blood of sequestration crisis ?
increased reticulocyte count
34
x
35
x
36
x
37
Sickle cell patients should be started on long term
hydroxycarbamide - reduce the incidence of complications and acute crises
