[microcyticanaemias] Flashcards
1
Q
A
Blood loss via menstruation
2
Q
A
hookworm (GI bleeding)
3
Q
A
coeliacs
4
Q
A
koilonychia
angular stomatitis
5
Q
A
poikilocytosis
6
Q
A
anisocytosis
7
Q
A
low (high TIBC)
8
Q
A
lack of iron means the precursor to haem (protoporphyrin 9) associated with a zinc iron.
9
Q
A
raised
10
Q
A
ferritin
11
Q
A
poor diet (rarely in adults)
12
Q
A
history of menorrhagia
13
Q
A
Ferrous sulphate (200mg PO)
14
Q
A
diarrhoea/constipation
nausea
discomfort abdominal
15
Q
A
until normal level reached. then for 3 months (replenish)
16
Q
A
investigate GI loss (colonoscopy, barium enema, gastroscopy)
17
Q
A
if hookworm ova suspected (tropics)
18
Q
A
microcytic anaemia unresponsive to iron
19
Q
A
sideroblasts with characteristic peri-nucleur ring of iron granules
20
Q
A
iron deposition
21
Q
A
increased
22
Q
A
Pyridoxine
23
Q
A
oral route not tolerated
functional iron deficiency (renal failure)
24
Q
A
High
High
25
[Thalassaemia]: what causes RBC rupture
unmatched globin chains precipitating, damaging membranes and rupture in the marrow
26
[Thalassaemia]: Point mutations in Ch.11 coding for the ... chain
beta-globin
27
[Thalassaemia]: name the 3 types of chain mutation combinations
B+/B+
B0/B0
B+/B0
*where 'B+' is decreased and 'B0' is no beta-globin*
28
[Thalassaemia]: what are the 3 main types of Hb in blood
HbA
HbA2
HbF
29
[Thalassaemia]: HbA in 97% of adult Hb, what chains is it composed of.
alpha2beta2
30
[Thalassaemia]: HbA2 in 2.5% of adult Hb, what chains is it composed of.
alpha2delta2
31
[Thalassaemia]: HbF in 90% of fetal Hb, what chains is it composed of.
alpha2gamma2
32
[Thalassaemia]: mild anaemia suggests what thalassaemia
trait aka minor
| Alpha2:Bnormal/B+
33
[Thalassaemia]: which thalassaemia is asymptomatic usually
minor
34
[Thalassaemia]: what might worsen Ttrait
pregnancy
35
[Thalassaemia]: what happens to the HbA2 in thalassaemia trait
increased (>3.5%)
36
[Thalassaemia]: when does Cooleys anaemia present
1st year
37
[Thalassaemia]: Observed in baby with thalassaemia major? (3)
failure to thrive
severe anaemia
extramedullary haematopoeisis (skull bossing)
38
[Thalassaemia]: X-ray on skull in Cooleys shows
'hair on end' due to increased marrow activity
39
[Thalassaemia]: which cardiac investigations would you do?
MRI
40
[Thalassaemia]: why would you wish to investiagate the heart?
Iron deposition
41
[Thalassaemia]: what investigations may you do? (7)
```
MCV
FBC
Iron
HbA2 (rises)
MRI
Film
Electrophoresis Hb
```
42
[Thalassaemia]: what does Hb electrophoresis discover?
Different variants of Hb (adult, fetal, A2)
43
[Thalassaemia]: What does siderosis mean?
Deposition of iron
44
[Thalassaemia]: how can you monitor for cardiac siderosis
MRI
45
[Thalassaemia]: why is there increased iron? (3)
Transfusions
increased iron uptake in gut
haemolysis
46
[Thalassaemia]: What may be present on examination as a result of haemolysis?
hepatosplenomegaly
47
[Thalassaemia]: Ix: what comments could be made from a blood film about the RBCs in Cooleys (3)
Hypochromic
Microcytic
Nucleated
48
[Thalassaemia]: Ix: When are target cells seen?
Abnormalities in Hb
49
[Thalassaemia]: Ix: What type of cell may be identifiable on Cooleys blood film?
target
50
[Thalassaemia]: What is the Tx for Major thalassaemia?
Lifelong transfusions (every 2-4 weeks)
51
[Thalassaemia]: how regular are the transfusions in Cooleys
2-4 weeks
52
[Thalassaemia]: apart from allowing normal growth why do we transfuse cooleys?
suppress abnormal bossing/extra-medullary growth
53
[Thalassaemia]: What level of Hb do you aim for with life long transfusions?
>90mg/L
54
[Thalassaemia]: what is the danger with life long transfusions
Siderosis/overload after 10 years
55
[Thalassaemia]: how does siderosis manifest endrocrinologically after 10 years of transfusions; give 5 examples.
```
pancreas --> DM)
Cardiac (reduced exercise tolerance)
Hypothyroidism
Hypocalcaemia
Hypogonadism
```
56
[Thalassaemia]: what bone complication is seen in Cooleys except for extramedullary growth?
Osteopenia
57
[Thalassaemia]: what is Tx for Osteopenia induced by extramedullary poeisis
Zoledronic acid
58
[Thalassaemia]: how does zoledronic acid work?
bisphosphonate
| Reduced osteoclastic activity
59
[Thalassaemia]: intermedia: define?
not requiring transfusions
| moderate anaemia
60
[Thalassaemia]: intermedia: apart from b+/b+ what other peptide chains are possible?
b+/ and another haemoglobinopathy e.g. sickle cell
61
[Thalassaemia]: Tx: what lifestyle advice should you give. (2)
healthy diet
| fitness/exercise
62
[Thalassaemia]: Tx: what supplements may you give?
Folate
63
[Thalassaemia]: Tx: why is folate useful?
deficiency due to reduced extreme demands by very active bone. Could progress to megaloblastic anaemia.
64
[Thalassaemia]: Tx: what drug is co-prescribed with transfusions to prevent unwanted SEs of transfusion.
Iron chelators
| Deferiprone + desferrioxamie sc 2xweekly
65
[Thalassaemia]: Tx: how is siderosis avoided? 2 drugs:
Deferiprone
+
desferrioxamie
SC
twice weekly
66
[Thalassaemia]: Tx: What infection are you at increased risk of with iron chelators
Yersinia
67
[Thalassaemia]: Tx: SEs of deferiprone and desferrioxamine (3)
cataracts
retinal damage
deafness
68
[Thalassaemia]: Tx: how can you increased urinary excretion of iron?
ascorbic acid
69
[Thalassaemia]: Tx: indications for splenectomy?
persistent hypersplenism with increasing transfusion requirements
70
[Thalassaemia]: Tx: after how old you wait before removing the spleen to avoid infections
5 years
71
[Thalassaemia]: Tx: is there a cure?
Bone marrow transplant
72
[Thalassaemia]: alpha thalassaemia: what genetic mutation causes alpha thal?
deletion
73
[Thalassaemia]: alpha thalassaemia: Barts Hydrops is what?
All 4 alpha genes deleted (a-a-/a-a-) therefore no alpha chains
74
[Thalassaemia]: alpha thalassaemia: what is the result of Barts hydrops
death in utero
75
[Thalassaemia]: alpha thalassaemia: if 3 alpha genes deleted what is the clinical presentation?
moderate anaemia
| haemolysis
76
[Thalassaemia]: alpha thalassaemia: what may be seen on blood film in --/-a ?
Beta tetramers
77
[Thalassaemia]: alpha thalassaemia: with 2 alpha genes deleted what is the clinical presentation
asymptomatic
78
[Thalassaemia]: alpha thalassaemia: if one alpha gene deleted what is the clinical state?
normal
79
[Thalassaemia]: alpha thalassaemia: with 2 alpha genes deleted what would be seen on film?
MCV reduced.
