Haemolysis Flashcards

1
Q

Define haemolysis:

A

Premature red blood cell destruction

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2
Q

List some reasons why RBCs are vulnerable to being damaged:

A

Their biconcave shape
No mitochondria = limited metabolic reserve
No nucleus

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3
Q

What is compensated haemolysis:

A

When the increased red blood cell destruction is beign compensated by increased RBC production
Therefore Hb levels normal

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4
Q

What is decompensated haemolysis?

A

Haemolytic anaemia
When the rate of RBC destruction exceeds the capacity of the bone marrow to produce RBCs
Low HB

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5
Q

State two consequences of haemolysis:

A

Erythroid hyperplasia

Excess RBC breakdown products e.g. bilirubin

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6
Q

What is the bone marrow’s response to haemolysis?

A

Reticulocytosis

Erythroid hyperplasia

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7
Q

Reticulocytes have a nucleus : true/false?

A

FALSE

They do not have a nucleus

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8
Q

How is haemolysis classified?

A

Extravascular i.e. RBCs being taken up by reticuloendothelial system (spleen / liver)
Intravascular i.e. red ells destroyed within the circulation

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9
Q

Which type of haemolysis is more common?

A

Extravascular

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10
Q

What might be seen on examination pf a patient with extravascular haemolysis?

A

Hyperplasia of site of destruction i.e. hepatomegaly or splenomegaly

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11
Q

What symptoms might be seen in extravascular haemolysis and why?

A

Jaundice & gallstones –> unconjugated bilirubin

Discoloured urine –> Urobilinogenuria

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12
Q

What is specific about the products seen in extravascular destruction?

A

They are normal blood components in excess

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13
Q

What is specific about the products seen in intravascular destruction?

A

Abnormal products seen in the blood.

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14
Q

List some products seen in the blood in intravascular red blood cell destruction?

A

Haemoglobinaemia (Free Hb in circulation)Methaemalbuminaemia
Haemoglobinuria (pink urine which turns black on standing)
Haemosiderinuria

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15
Q

List some causes of intravascular haemolysis:

A

ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria
Rare (PNH / PCH)

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16
Q

What investigations are done for haemolysis:

A
FBC and film 
Reticulocyte count
Serum unconjugated bilirubin
Serum haptoglobins
Urinary urobilinogen
17
Q

What might be seen on a blood film of a patient with haemolysis?

A

Spherocytes
Red cell fragments
Heinz bodies
Sickle cells

18
Q

What is a specific investigation for haemolysis?

A

Direct Coombs test

19
Q

What antibodies are associated with autoimmune haemolysis?

A

Warm - IgG

Cold - IgM

20
Q

List some causes of warm IgG

A
Idiopathic
Autoimmune e.g. SLE
Lymphoproliferative 
Drugs e.g. penicillin
Infections
21
Q

List some causes of Cold IgM

A

Idiopathic
Infections e.g. EBV, mycoplasma
Lymphoproliferative disorders

22
Q

Outline the mechanism of the direct coombs test:

A

Patients RBCs are mixed with mouse antibodies coated with human anti-IgG and complement
If patient is positive, blood will agglutinate

23
Q

Give some examples of alloimmune haemolysis:

A

Immune response e.g. haemolytic transfusion reaction (immediate IgM = intravascular and delayed IgG = extravascular)

Passive transfer of antibody e.g. haemolytic disease of the newborn

24
Q

List some causes of mechanical red cell destruction:

A
Disseminated intravascular coagulation
Haemolytic uraemic syndrome 
TTP
Leaking heart valve
Infections e.g. malaria
25
Q

What bacteria causes HUS?

A

E. Coli 0157

26
Q

What type of anaemia is associated with a mechanical heart valve and what is seen on the blood film?

A

Microangiopathic haemolytic anaemia
(MAHA)

Red cell fragmentation due to mechanical damage

27
Q

When are microspherocytes seen in haemolytic anaemias?

A

When caused by severe burn injuries

RBCs are sheared when passing through damaged capillaries

28
Q

List some causes of membrane defects that lead to haemolysis:

A
Liver disease (Zieve's syndrome)
Vit E deficiency
Paroxysmal Nocturnal Haemoglobinuria
29
Q

What is Zieve’s syndrome?

A

An acute metabolic disorder which results from withdrawal after prolonged exposure to alcohol.
Defined by haemolytic anaemia, hyperlipidaemia and jaundice

30
Q

What genetic condition can cause abnormal RBC membranes, leading to haemolysis?

A

Hereditary Spherocytosis

31
Q

What does a G6PD deficiency cause?

A

Leads to failure to cope with oxidative stress and a failure to produce ATP, causing the metabolism process to fail

32
Q

How is spherocytosis inherited?

A

Dominant