Haemolysis Flashcards

1
Q

Why are red cells susceptible to damage?

A

Biconcave shape
Limited metabolic reserve and rely on glycolysis
Cant generate new proteins

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2
Q

What are the characteristics of compensated haemolysis?

A

Increased red cell destruction compensated by increased red cell production
Haemoglobin maintained

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3
Q

What are the characteristic of decompensated haemolysis?

A

Increased rate of red cell destruction
Exceeding capacity for red cell production
Hb falls

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4
Q

What are the consequences of haemolysis?

A

Erythroid hyperplasia

Excess red cell breakdown

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5
Q

What are the bone marrow responses to haemolysis?

A

Reticulocytosis

Erythroid hyperplasia

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6
Q

Are reticulocytes nucleated?

A

No

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7
Q

What do reticulocytes show?

A

Response to bleeding

Response to iron therapy

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8
Q

What stain shows reticulocytes?

A

New methylene blue stains ribosomal RNA

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9
Q

What is extravascular haemolysis?

A

Taken up by reticuloendothelial system by liver and spleen

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10
Q

What is intravascular haemolysis?

A

Red cells destroyed within the circulation

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11
Q

WHat is commoner Extravascular or intravascular haemolysis?

A

Extra

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12
Q

What is seen in extravascular haemolysis?

A

Hyperplasia at destruction site (splenomegaly, hepatomealy)

Release of protoporphyrin

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13
Q

What is seen due to release of protoporphyrin?

A
Unconjugated bilirubinaemia
Jaundice
Gall stones
Urobilinogenuria
Normal products in excess
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14
Q

What is seen in intravascular haemolysis?

A
Haemoglobinaemia
Methaemalbuminaemia
Haemoglobulinuria
Haemosiderinuria
Abnormal products
LIFE THREATENING
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15
Q

Which is life threatening Extra or intravascular haemolysis?

A

Intravascular

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16
Q

What can cause intravascular haemolysis?

A

ABO incompatibility
G6PD deficiency
Severe falciparum malaria
PNH PCH

17
Q

What investigations are doen for haemolysis?

A
Confrim haemolytic state
-FBC
-Reticulocute count
-Serum Unconjugated bilirubin
-Serum haptoglobins
-Urinary urobilinogen
Identify cause:
-History and examination
Blood film 
Coombs test
18
Q

What are 4 sites of red cell defect?

A

Premature destruction of normal red cells
Abnormal cell membrane
Abnormal red cell metabolism
Abnormal haemoglobin

19
Q

What are causes of IgG autoimmune haemolysis?

A
Idiopathic 
Autoimmune disorders
Lymphoproliferative disorders
Drugs
Infections
20
Q

What are causes of IgM autoimmune haemolysis?

A

Idiopathic
Infections
lymphoproliferative disorders

21
Q

What is the purpose of direct coombs test?

A

Identify antibody bound to own red cells

patients red cells with anti-human IgG

22
Q

What are causes of alloimmune haemolysis?

A

Immune response

Passive transfer of antibody

23
Q

What is an example of immune response in alloimmune haemolysis?

A

Haemolytic transfusion reaction
Immediate IgM intravascular
Delayed igG extravascular

24
Q

What is an example of Passive transfer of antibody?

A

Haemolytic disease of the newborn
RhD
ABO incompatibility

25
What can cause mechanical red cell destruction?
``` Disseminated intravascular coagulation Haemolytic uraemic syndrome TTP Leaky valve Infections (Malaria) ```
26
What can cause membrane defects?
Liver disease Vitamin E deficiency Paroxysmal nocturnal haemoglobinuria
27
What is Zieve's syndrome?
Haemolysis Alcoholic liiver disease Hyperlipidaemia
28
What can cause genetic red cell membrane abnormalities?
Reduced membrane deformabilitu Increased transit time through spleen Oxidant environment in spleen causing extravascular red cell destruction Hereditary spherocytosis
29
What can be deficient and lead to reduced ATP production?
G6PD deficiency | Metabolic process fail
30
What drugs can cause oxidative damage?
Dapsone | Salazopyrin
31
What can cause abnormal haemoglobin?
Sickle cell disease