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T IoD haemaology > haemolytic anaemias and haemoglobinopathies > Flashcards

haemolytic anaemias and haemoglobinopathies Flashcards

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.

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2
Q

where is the pathology in haemolytic anaemia?

A

In the red blood cells or it’s environment

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3
Q

What are examples of inherited haemolysis?

A

Sickle cell disease
membrane disorders i.e hereditary spherocytosis
G6PDdeficiency

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4
Q

what are example of acquired haemolysis?

A

paroxysmal nocturnal haemoglobinueia
valve haemolysis
autoimme warm or cold

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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
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6
Q

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

A

DAT

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7
Q

what is the difference between intravascular and extravascular haemolysis?

A

Intravascular Is in blood vessels

Extravascular is in the lymph and spleen

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8
Q

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

A

unconjugated bilirubin increased
increased LDH
increased reticulocytes

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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

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10
Q

what are possible auto immune acquired haemolytic anaemias?

A

Warm
Cold
Drug induced

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11
Q

what are examples of allo immune haemolytic anaemias?

A

transfusion reactions

haemolytic disease of the foetus and newborns

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12
Q

what are the main mediators in warm autoimmune haemolysis?

A

IgG

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13
Q

what are the main mediators in cold autoimmune haemolysis?

A

IgM

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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

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15
Q

what DAT results does warm autoimmune haemolytic anaemia normally give?

A

positive

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16
Q

Is warm AIHA normally intravascular or extravascular?

A

extravascular

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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

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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

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19
Q

what treatments can be given for warm autoimmune haemolytic anaemia?

A

Steroids
Blood
rituximab
splenectomy

20
Q

what is microangiopathic haemolytic anaemia?

A

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

21
Q

what is thrombotic thrombocytopenic purpura?

A

A type of microangiopathic haemolytic anaemia.

22
Q

what is the pentad seen in thrombotic thrombocytopenic purpura?

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

what are causes of thrombotic thrombocytopenic purpura?

A

often idiopathic

can be HIV, pregnancy, drugs, congenital

24
Q

why is there aggregation in thrombotic thrombocytopenic purpura?

A

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

25
Q

what is ADAMTS 13 and how does it change in thrombotic thrombocytopenic purpura?

A

A protease which breaks down vWF multimers

In TTP it’s reduced contributing to the aggregation

26
Q

what is disseminated intravascular coagulopathy?

A

A type of microangiopathic haemolytic anaemia

27
Q

what is the process in disseminated intravascular coagulopathy?

A

There is systemic activation of coagulation pathways, fibrin clots form causing organ failure
there is consumption of platelets and factors causing bleeding

28
Q

how is disseminated intravascular coagulopathy diagnosed?

A

Increased PT,APTT and D Dimer

reduced fibrinogen and platelet count

29
Q

what is the defect in paroxysmal nocturnal haemoglobinuria?

A

a PIG-A defect causing reduced GPI proteins (CD55 AND CD59)

30
Q

in paroxysmal nocturnal haemoglobinuria CD59 is reduced, what does CD59 normally do?

A

protects against MAC to prevent complement mediated lysis

31
Q

when does homozygous sickle cell anaemia normally present?

A

In early childhood with anaemia and jaundice

32
Q

what symptoms are seen in homozygous sickle cell anaemia?

A 
Painful hands and feet
inflammation of fingers
pleuritic chest pain
SOB
hypoxia
33
Q

what are some causes of homozygous sickle cell anaemia episode?

A 
dehydration
infection
cold/damp conditions
unaccustomed exercise
stress
pregnancy
34
Q

People with homozygous sickle cell anaemia can have a chest crisis how is this managed?

A

Fluids
oxygen
analgesia
long term hydroxyurea

35
Q

what is haemoglobinopathy?

A

A single gene disorder due to an abnormality in the globin chain structure

36
Q

what causes thalassaemias?

A

Caused by absent or reduced production of the alpha and beta globin chains caused by mutations in regulatory genes

37
Q

what is the genetic change in sickle cell disease?

A

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
Q

what is the pathophysiology in sickle cell disease?

A

The oxygenated RBC contains HbS, when deoxygenated the HbS polymerises causing the sickling shape.
This can cause occlusion leading to infarct

39
Q

why is there hypercoagubility in sickle cell disease?

A

there is inflammation causing increased expression of VCAM 1 and other adhesion molecules

40
Q

what are chronic complications of sickle cell?

A 
Silent infarcts
pulmonary hypertension
erectile dysfunction
chronic pain syndrome
delayed puberty
retinopathy
visual loss
chronic renal loss
avascular necrosis
41
Q

what is the pain management in sickle cell?

A

quick analgesia and review in half an hour
believe the patient
hydration
VTE prophylasix

42
Q

what is the result of ao homozygous thalassaemia?

A

fetal death or hydrops fetalis

43
Q

what is the result of alpha positive heterozgote thalassaemia?

A

some alpha chain production meaning normal HbA and HbB4 production

44
Q

what is the result of Beta o homozygote thalassaemia?

A

severe haemolytic disease

45
Q

what is the result of beta positive heteroygote thalassaemia?

A

symptomless

T IoD haemaology (6 decks)

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