Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

T IoD haemaology > haemolytic anaemias and haemoglobinopathies > Flashcards

haemolytic anaemias and haemoglobinopathies Flashcards

(45 cards)

Start Studying
1
Q

what is haemolytic anaemia?

A

there is increased destruction of RBC’s so they have a shorter half life, but the BM works harder to produce more.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

where is the pathology in haemolytic anaemia?

A

In the red blood cells or it’s environment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are examples of inherited haemolysis?

A

Sickle cell disease
membrane disorders i.e hereditary spherocytosis
G6PDdeficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what are example of acquired haemolysis?

A

paroxysmal nocturnal haemoglobinueia
valve haemolysis
autoimme warm or cold

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what results will you see in a abnormal haemolysis screen?

A
  • FBC; normocytic anaemia
  • Blood film: spherocytes of red cell fragments
  • Reticuloyctes: increased
  • LDH; increased due to increased cell turnover
  • bilirubin: increased unconjugated
  • Haptoglobin decreased
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what test can be done to find out if the haemolysis is immune or not?

A

DAT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the difference between intravascular and extravascular haemolysis?

A

Intravascular Is in blood vessels

Extravascular is in the lymph and spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what results are the same in both intravascular and extravascular anaemia?

A

unconjugated bilirubin increased
increased LDH
increased reticulocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what results are different in intravascular and extravascular haemolysis?

A 
Intravascular:
decreased haptoglobin
haemoglobinuria
haemoglobinaemia
hemosiderinuria

extravascular: normal haptoglobin
no haemoglobineria
no haemoglobinaemia
no hemosiderinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are possible auto immune acquired haemolytic anaemias?

A

Warm
Cold
Drug induced

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are examples of allo immune haemolytic anaemias?

A

transfusion reactions

haemolytic disease of the foetus and newborns

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are the main mediators in warm autoimmune haemolysis?

A

IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are the main mediators in cold autoimmune haemolysis?

A

IgM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

how is coombs test (DAT) carried out?

A

take a blood sample, the patients RBC’s are washed and incubated with antihuman antibodies.
RBC’s agglutinate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what DAT results does warm autoimmune haemolytic anaemia normally give?

A

positive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Is warm AIHA normally intravascular or extravascular?

A

extravascular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

If there is a child playing outside and then come in the warm and experience haemolysis what is the condition?

A

paroxysmal coldhaemoglobinuria

binds to RBC’s at 37 degreases and lyses at 20 degrees

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the difference on blood film between warm and cold autoimmune haemolytic anaemia?

A

Warm: slightly darker and smaller red cells
no central pallow
spherocytes

cold:
Agglutination so bunches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what treatments can be given for warm autoimmune haemolytic anaemia?

Study These Flashcards
A

Steroids
Blood
rituximab
splenectomy

20
Q

what is microangiopathic haemolytic anaemia?

Study These Flashcards
A

There is mechanical destruction of red blood cells and schistocytes on the film

21
Q

what is thrombotic thrombocytopenic purpura?

Study These Flashcards
A

A type of microangiopathic haemolytic anaemia.

22
Q

what is the pentad seen in thrombotic thrombocytopenic purpura?

Study These Flashcards
A
  1. fever
  2. renal failure
  3. confusion
  4. thrombocytopenia
  5. MAHA blood film finding
23
Q

what are causes of thrombotic thrombocytopenic purpura?

Study These Flashcards
A

often idiopathic

can be HIV, pregnancy, drugs, congenital

24
Q

why is there aggregation in thrombotic thrombocytopenic purpura?

Study These Flashcards
A

There is endothelial cell damage which releases ultra large vWF multimers which then bind to receptors on platelets

25
what is ADAMTS 13 and how does it change in thrombotic thrombocytopenic purpura?
A protease which breaks down vWF multimers | In TTP it's reduced contributing to the aggregation
26
what is disseminated intravascular coagulopathy?
A type of microangiopathic haemolytic anaemia
27
what is the process in disseminated intravascular coagulopathy?
There is systemic activation of coagulation pathways, fibrin clots form causing organ failure there is consumption of platelets and factors causing bleeding
28
how is disseminated intravascular coagulopathy diagnosed?
Increased PT,APTT and D Dimer | reduced fibrinogen and platelet count
29
what is the defect in paroxysmal nocturnal haemoglobinuria?
a PIG-A defect causing reduced GPI proteins (CD55 AND CD59)
30
in paroxysmal nocturnal haemoglobinuria CD59 is reduced, what does CD59 normally do?
protects against MAC to prevent complement mediated lysis
31
when does homozygous sickle cell anaemia normally present?
In early childhood with anaemia and jaundice
32
what symptoms are seen in homozygous sickle cell anaemia?
``` Painful hands and feet inflammation of fingers pleuritic chest pain SOB hypoxia ```
33
what are some causes of homozygous sickle cell anaemia episode?
``` dehydration infection cold/damp conditions unaccustomed exercise stress pregnancy ```
34
People with homozygous sickle cell anaemia can have a chest crisis how is this managed?
Fluids oxygen analgesia long term hydroxyurea
35
what is haemoglobinopathy?
A single gene disorder due to an abnormality in the globin chain structure
36
what causes thalassaemias?
Caused by absent or reduced production of the alpha and beta globin chains caused by mutations in regulatory genes
37
what is the genetic change in sickle cell disease?
a point mutation in the beta globin gene on chromosome 11 causing a substation of adenine to thrmine meaning valine replaces glutamic acid at position 6
38
what is the pathophysiology in sickle cell disease?
The oxygenated RBC contains HbS, when deoxygenated the HbS polymerises causing the sickling shape. This can cause occlusion leading to infarct
39
why is there hypercoagubility in sickle cell disease?
there is inflammation causing increased expression of VCAM 1 and other adhesion molecules
40
what are chronic complications of sickle cell?
``` Silent infarcts pulmonary hypertension erectile dysfunction chronic pain syndrome delayed puberty retinopathy visual loss chronic renal loss avascular necrosis ```
41
what is the pain management in sickle cell?
quick analgesia and review in half an hour believe the patient hydration VTE prophylasix
42
what is the result of ao homozygous thalassaemia?
fetal death or hydrops fetalis
43
what is the result of alpha positive heterozgote thalassaemia?
some alpha chain production meaning normal HbA and HbB4 production
44
what is the result of Beta o homozygote thalassaemia?
severe haemolytic disease
45
what is the result of beta positive heteroygote thalassaemia?
symptomless

T IoD haemaology (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions