[sicklecell] Flashcards Preview

HAEMATOLOGY > [sicklecell] > Flashcards

Flashcards in [sicklecell] Deck (41):
1

[sicklecell]: is an autosomal recessive leading to malproduction of the ... chains in Hb

Beta

2

[sicklecell]: what is the amino acid change at position 6

Glutamic acid --> valine

3

[sicklecell]: HbA should be produced however ... is instead

HbS

4

[sicklecell]: in what ethnicity is it common

africans

5

[sicklecell]: what is the genotype for Sickle cell trait

heterozygotes (HbAS)

6

[sicklecell]: HbSS is trait or anaemia

anaemia

7

[sicklecell]: .... protect against falciparum

heterozygote (HbAS)

8

[sicklecell]: trait is asymptomatic in most circumstances. When might vaso-occlusive crisis occur

hypoxia
(anaethesia/enclosed spaces etc)

9

[sicklecell]: what is a vaso-occlusive crisis

vessels occluded by build up of sickle cells leading to painful ischaemia

10

[sicklecell]: symptomatic trait sickling occurs in comjuction with other beta globinopathies like (2)

thalassaemia
HbC

11

[sicklecell]: why do the RBCs 'sickle'

polymerisation of deformed HbS when deoxygenated

12

[sicklecell]: why does haemolysis occur?

sickle cells are fragile

13

[sicklecell]: what would be seen on blood film (2)

sickle cells
Target cells

14

[sicklecell]: what is the gold standard Dx

Hb electrophoresis

15

[sicklecell]: why is Hb electrophoresis the gold Dx?

allows distinction between HbSS and HbAS

16

[sicklecell]: when should a person be Dx with SC?

at birth (cord blood)

17

[sicklecell]: why do you Dx someone at birth with SC

pneumococcal prophylaxis

18

[sicklecell]: at birth why prophylactic drugs are given?

sickle cell reduces spleen function i.e.immunocompromised

19

[sicklecell]: acute abdomen in SC is likely to be seen durin a ....

vaso-occlusive crisis

20

[sicklecell]: mesenteric ischaemia as a result of vaso-occlusive crisis can mimic an ...

acute abdomen

21

[sicklecell]: why is there an increased risk of CNS stoke/TIA/seizures

vaso-occlusive crisis

22

[sicklecell]: how can risk of stroke be measured

doppler US

23

[sicklecell]: vaso-occlusive crises especially affect the ...

bone
(e.g. avascular necrosis of femoral head)

24

[sicklecell]: vaso-occlusive crisis affect the male genitalia in what way

priapism

25

[sicklecell]: which virus is associated with an aplastic crisis

parvovirus b19

(invades and destroys reticulocytes)

26

[sicklecell]: An aplastic crisis results in a reduction in

especially RBCs

27

[sicklecell]: why do sequestration crises usally affect children

the spleen hasnt undergone atrophy

28

[sicklecell]: what is a sequestration crisis

intrasplenic trapping of RBCs usually affecting children

29

[sicklecell]: what is the main acute medical emergency associated with SC

sequestration crisis

death in 1-2 hours due to circulatory failure

30

[sicklecell]: 40% of SC deaths are caused by .... due to splenic occlusion

infection

31

[sicklecell]: Tx: 1st line in an acute crisis?

hydroxycarbamide

32

[sicklecell]: Tx: what is the MOA of the 1st line drug used in an acute crisis?

increases the level of HbF

33

[sicklecell]: Tx: only curative treatment?

bone marrow transplant

34

[sicklecell]: Tx: for sequestration crisis

urgent transfusion

35

[sicklecell]: CRISIS: in which crisis do patients get prodromal painful crisis 2.5 days before?

acute chest syndrome

36

[sicklecell]: CRISIS: what is the pathophysiology behind

pulmonary infiltrates

37

[sicklecell]: CRISIS: acute chest syndrome symptoms (3)

tachypnoea
pain
fever

38

[sicklecell]: CRISIS: what is the Tx for acute chest syndrome (4)

O2
analgesia
emirical antibiotics
transfusion if severe (X-match)

39

[sicklecell]: CRISIS: what is the most common infiltrates into the lung in acute chest syndrome (2)

fat embolism
infection

40

[sicklecell]: CRISIS: what are the most common infectiour organisms in acute chest syndrome (2)

chlamydia
mycoplasma

41

[sicklecell]: Tx: what is the indication for blood transfusion? (3)

severe chest crisis
CNS event
multiorgan failure