Haemolysis Flashcards

1
Q

what is haemolysis

A

Premature red cell destruction

i.e. shortened red cell survival

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2
Q

why are red cells susceptible to damage

A
  1. need a biconcave shape to transit the circulation
  2. limited metabolic reserve and rely on glucose metabolism for energy (no mitochondria)
  3. Can’t generate new proteins once in the circulation (no nucleus)
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3
Q

what is compensated haemolysis

A

Increased red cell destruction compensated by increased red cell production
i.e. Hb Maintained

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4
Q

what is decompensated haemolysis also known as

A

Haemolytic anaemia

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5
Q

what is haemolytic anaemia

A

Increased rate of red cell destruction exceeding bone marrow capacity for red cell production
i.e. Hb Falls

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6
Q

what are consequences of haemolysis

A

Erythroid hyperplasia (increased bone marrow red cell production)

Excess red cell breakdown products eg billirubin (clinical features differ by aetiology and site of red cell breakdown)

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7
Q

what is the bone marrow response to haemolysis

A

1 - reticulocytosis

2 - erythroid hyperplasia [see a higher number of precursors of RBC in the bone marrow]

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8
Q

what special stain can be used to just see reticulocytes on a blood film

A

supravital stain

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9
Q

how can haemolytic anaemia be classified

A

extravascular
- RBC destroyed by spleen and liver

intravascular
- RBC destroyed within the circulation

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10
Q

what form of haemolytic anaemia is more common

A

extravascular

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11
Q

what are features of extravascular haemolytic anaemia

A

splenomegaly +/- hepatomegaly

Release of protoporphyrin

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12
Q

what does release of protoporphyrin cause

A

Unconjugated bilrubinaemia
> Jaundice and Gall stones

Urobilinogenuria

Normal products

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13
Q

what does it mean if bilirubin is unconjugated

A

pre hepatic cause

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14
Q

what is the pathophysiology of intravascular haemolysis

A

Red cells are destroyed in the circulation spilling their contents.

  1. Haemoglobinaemia (free Hb in circulation)
  2. Methaemalbuminaemia
  3. Haemoglobinuria: pink urine, turns black on letting the urine stand
  4. Haemosiderinuria
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15
Q

what is the big difference in symptoms between extra and intra vascular haemolysis

A

intravascular cause ABNORMAL products

- may be life threatening

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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

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17
Q

what Ix are done first in haemolytic anaemia

A
FBC (+ BLOOD FILM)
Reticulocyte count
Serum unconjugated bilirubin
Serum haptoglobins
Urinary urobilinogen
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18
Q

what Ix are done second in haemolytic anaemia

A
Blood Film: 
Membrane damage (spherocytes)
Mechanical damage (red cell fragments)
 Oxidative damage (Heinz bodies)
 Others e.g.. HbS (sickle cells)
19
Q

what is another way to classify haemolytic anaemia

A

by site of red cell defect

20
Q

what are the 4 types of haemolytic anaemia by site

A
  1. Premature destruction of normal red cells (immune or mechanical)
  2. Abnormal cell membrane
  3. Abnormal red cell metabolism
  4. Abnormal haemoglobin
21
Q

what are acquired immune haemolysis

A

autoimmune haemolysis

alloimmune haemolysis

22
Q

what are the subtypes of autoimmune haemolysis

A

warm (IgG)

cold (IgM)

23
Q

what are the cause of warm autoimmune haemolysis

A

idiopathic
autoimmune disorders (SLE)
Lymphoproliferative disorders (CLL)
Drugs (penicillins, etc) Infections

24
Q

what is the test for autoimmune haemolytic anaemia

A

direct Coombs test

25
Q

what are the two possible forms of alloimmune haemolysis

A

immune response (antibody produced)

passive transfer of antibody

26
Q

what can cause an immune response leading to haemolysis

A

Haemolytic transfusion reaction

  • Immediate (IgM) predominantly intravascular
  • Delayed (IgG) predominantly extravascular
27
Q

what can cause an passive transfer of antibody leading to haemolysis

A

Haemolytic disease of the newborn

28
Q

what are causes of mechanical red cell destruction

A
DIC
HUS
TTP 
leaking heart valve 
infections e.g. malaria
29
Q

what seen in a blood film would be suggestive of a mechanical red cell destruction

A

red cell fragmentation

30
Q

what is seen in burns related haemolysis

A

Microspherocytes

- small spherocytes predominate and red blood cells are smaller and more globular than normal

31
Q

what are causes of acquired RBC membrane defects (rare)

A
Liver Disease (Zieve’s Syndrome)
Vitamin E deficiency
Paroxysmal Nocturnal Haemoglobinuria
32
Q

what is Zieve’s syndrome

A

Haemolysis, Alcoholic liver disease, hyperlilidaemia

33
Q

what are causes of congenital RBC membrane defects

A

Hereditary Spherocytosis

34
Q

what are genetic causes of abnormal red cell metabolism

A

failure to cope with oxidant stress (G6PD deficiency - most common form)

failure to generate ATP: metabolic processes fail

35
Q

what can cause stress to the metabolic pathways in normal cells

A

dapsone or salazopyrin

36
Q

what does dapsone cause

A

bite cells a.k.a irregularly contracted cell

37
Q

what genetic disease can cause haemolytic anaemia by making abnormal haemoglobin

A

Sickle cell disease

38
Q

what are pappenheimer bodies and what are they seen in

A

abnormal granules of iron found inside red blood cells

beta thalassaemia major

39
Q

what type of haemolysis is seen in hereditary spherocytosis

A

Chronic extravascular haemolysis.

40
Q

what does the defect affect in hereditary spherocytosis

A

the RBC membrane flexibility

41
Q

what type of haemolysis is seen in Falciparum malaria

A

intravascular haemolysis

42
Q

what is seen on a blood film of HbH

A

inclusion bodies

- golf ball appearance

43
Q

what are Heinz bodies

A

Red cell inclusions comprised of denatured Hb normally removed by spleen (eg seen in G6PD deficiency)