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Haematology > Haemolysis > Flashcards

Haemolysis Flashcards

1
Q

What is the definition if haemolysis?

A

Pemature red cell destruction

i.e. shortened red cell survival

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2
Q

What 3 reasons mean red cells are susceptible to damage?

A
  1. They need biconcave shape
  2. Limited metabolic reserve (no mitochondria)
  3. Can’t generate new proteins once in circulation (no nucleus)
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3
Q

What is compensated haemolysis?

A

Increased red cell destruction compensated by increased red cell production

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4
Q

What is haemolytic anaemia (decompensated haemolysis)?

A

Increased rate of red cell destruction exceeding bone marrow capacity for red cell production

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5
Q

What are consequences of haemolysis?

A

Erythroid hyperplasia

Excess red cell breakdown products e.g. biliruben

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6
Q

What is Erythroid hyperplasia?

A

Increased bone marrow red cell production

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7
Q

What is the bone marrow response to haemolysis?

A

Reticulocytosis

Erythroid hyperplasia

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8
Q

Are reticulocytes nucleated ells?

A

No

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9
Q

What is ribosomal RNA labelled with to count flourescent cells?

A

Flourochrome

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10
Q

What is extravascular haemolysis?

A

Red cells taken up by reticuloendothelial system (liver and spleen mainly)

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11
Q

What is intravascular haemolysis?

A

Red cells destroyed within circulation

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12
Q

Describe extravascular red cell destrucion?

A

Commoner
Hyperplasia at site of destruction
Release of protoporphyrin

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13
Q

Which is more likely to be life threatening, intra or extra vascular haemolysis?

A

Intra

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14
Q

Why is intravascular so bad?

A

Red cells spill their contents into circulation

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15
Q

What are the 4 potential problems that can arise from intravascular haemolysis?

A
  1. Haemoglobinaemia (free Hb in circulation)
  2. Methaemalbuminaemia
  3. Haemoglobinuria (pink urine, turns black on standing)
  4. Haemosiderinuria
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16
Q

What are causes of intravascular haemolysis?

A

ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria (blackwater fever)
PNH,PCH (rare)

17
Q

What are causes of extravascular haemolysis?

A

Pretty much all other causes of haemolysis

18
Q

What are investigations for haemolysis?

A 
Confirm haemolytic state.
FBC (+blood film)
Reticulocyte count
Serum unconjugated biliruben
Serum haptoglobins
Urinary urobilinogen
19
Q

Name 4 things that should be looked for on blood film?

A

Red cell fragments
Spherocytes
Heinz bodies
Sickle cells

20
Q

Name 1 specialist test for haemolysis?

A

Direct Coombs’ test

21
Q

What are 4 classifications based on site of red cell defect?

A
  1. Premature destruction of normal red cells (immune or mechanical)
  2. Abnormal cell membrane
  3. Abnormal red cell metabolism
  4. Abnormal haemoglobin
22
Q

What are two types of autoimmune haemolysis?

A

Warm (IgG)

Cold (IgM)

23
Q

What are types of warm (IgG)?

A 
Idiopathic
Autoimmune (SLE)
Lymphoprolifrative disorders (CLL)
Drugs (penicillin)
Infections
24
Q

What are types of cold (IgM)?

A

Idiopathic
Infections (EBV, Mycoplasma)
Lymphoproliferative disorders

25
Q

What does a Coomb’s test do?

A

Identifies antibody (and complement) bound to own red cells

26
Q

What are two types of Alloimmune haemolysis?

A 
Immune response (antibody produced)
Passive transfer of antibody
27
Q

What is Immune response alloimmune haemolysis?

A

Haemolytic tranfusion reaction
Immeadiate, (IgM) predominantly intravascular
Delayed (IgG) predominantly extravascular

28
Q

What is passive transfer to antibody alloimmune haemolysis?

A

Haemolytic disease of the newborn
RhD
ABO incompatibility
Others e.g. anti-Kell

29
Q

What are causes of acquired haemolysis?

A 
Disseminated intravascular coagulation
Haemolytic uraemic syndrome (e.g. E.coli 0157)
TTP
leaking heart valve
Infectious e.g. malaria
30
Q

What is MAHA?

A

Mechanical valve related Microangiopathic haemolytic anaemia - red cell fragmentation as a result of mechanical (extrinsic damage)

31
Q

When are microspherocytes seen?

A

In burns related haemolysis

32
Q

What are membrane defect causes of haemolysis?

A 
Liver disease (Zieve's syndrome)
Vitamin E deficiency
paroxysmal Nocturnal haemoglobinuria
33
Q

What is seen in Zieve’s syndrome?

A

Anameia
polychromatic macrocytes
Irregular contracted cells

34
Q

What are genetic causes of haemolysis?

A

Reduced membrane deformability
Increased transit time through spleen
Oxidant environment in spleen causes extravascular red cell destruction
Hereditary spherocytosis

Haematology (27 decks)

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