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Haematology Year 3 > Haemolysis > Flashcards

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 in 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 know 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

consequences of haemolysis

A 
erythroid hyperplasia (increased bone marrow red cell production)
excess red cell breakdown products e.g. bilirubin
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7
Q

what is the bone marrow response to haemolysis

A

reticulocytosis

erythroid hyperplasia- see higher number of RBC precursors in the bone marrow

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8
Q

what stain is used to just see reticulocytes on a blood film

A

supravital stain

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9
Q

what is seen on blood film in reticulocytosis

A

polychomasia (blue staining ribosomal RNA)

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10
Q

how can haemolytic anaemia be classified

A

extravascular
- RBC destroyed by liver and spleen

Intravascular
- RBC destroyed within the circulation

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11
Q

what form of haemolytic anaemia is more common

A

extravascular

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12
Q

features of extravascular haemolysis

A

splenomegaly +/- hepatomegaly

release of protoporphyrin

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13
Q

effects of protoporphyrin release

A

Unconjugated bilrubinaemia – jaundice + gall stones

Urobiliogenuria

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14
Q

what does it mean if bilirubin is unconjugated

A

pre-hepatic cause

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15
Q

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 Turing, turns black on standing)
  4. Haemosiderinuria

ABNORMAL product

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16
Q

big difference between symptoms of extra and intra vascular haemolysis

A

intravascular causes ABNORMAL products- may be life threatening

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17
Q

cause of intravascular haemolysis

A

ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria (blackwater fever)
Paroxsysmal nocturnal haemoglobinuria (PNH)
Paroxsysmal cold haemoglobinuria (PCH)

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18
Q

investigations of haemolytic anaemia

A 
FBC + Blood film 
reticulocyte count
serum unconjugated bilirubin 
serum hapatoglobins
urinary urobilinogen
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19
Q

what is seen on blood film if there has been membrane damage

A

spherocytes

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20
Q

what is seen on blood film if there has been mechanical damage

A

red cell fragments

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21
Q

what is seen on blood film if there has been oxidative damage

A

Heinz bodies

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22
Q

what is another way to classify haemolytic anaemia

A

site of red cell defect

23
Q

what are the 4 types of haemolytic anaemia by 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 Hb
24
Q

causes of acquired haemolysis

A

immune:
autoimmune
alloimmune
(premature destruction of normal red cells)

25
subtypes of autoimmune haemolysis
warm (IgG) | cold (IgM)
26
What antibody is involved in warm autoimmune haemolysis
IgG
27
what antibody is involved in cold autoimmune haemolysis
IgM
28
causes of warm autoimmune haemolysis
``` Idiopathic (most common) autoimmune disorders (SLE) lymphoproliferative disorders (CLL) drugs (penicillin) infections ```
29
causes of cold autoimmune haemolysis
idiopathic infections (EBV, Mycoplasma) Lymphoproliferative disorders
30
test for autoimmune haemolytic anaemia
direct Coomb's test
31
two forms of alloimmune haemolysis
immune response (antibody produced) passive transfer of antibody
32
what can cause an immune response leading to alloimmune haemolysis
Haemolytic transfusion reaction: IgM : immediate (intravascular) IgG: delayed (extravascular)
33
what can cause a passive transfer of antibody leading to haemolysis
haemolytic disease of the new born
34
causes of mechanical red cell destruction
``` disseminated intravascular coagulation haemolytic uraemia syndrome (e.g. caused by E.Coli O157) TTP leaking heart valve malaria ```
35
what is seen in a blood film if there has been mechanical red cell destruction
red cell fragmentation
36
what is seen in a blood film if there has been burns related haemolysis
microspherocytes- small spherocytes predominate and RBCs are smaller and more globular than normal
37
causes of acquired RBC membrane defects (rare)
Liver disease -- Zieve's syndrome Vitamin E deficiency Paroxysmal nocturnal haemoglobinuria
38
what is Zieve's syndrome
Haemolysis, alcoholic liver disease, hyperlipidaemia
39
causes of congenital RBC membrane defect
hereditary spherocytosis
40
genetic causes of abnormal red cell metabolism
failure to cope with oxidant stress (G6PD deficiency is most common) failure to generate ATP: metabolic process fail
41
what can cause stress to metabolic pathways in normal cells
dapsone or salazopyrin
42
what does dapsone cause
bite cells a.ka. irregularly contracted cell
43
what genetic disease can cause haemolytic anaemia by making abnormal haemoglobin
sickle cell disease
44
what are pappenheimer bodies | what condition are they seen in
abnormal granules of iron found inside red blood cells | beta thalassaemia major
45
what type of haemolysis is seen in hereditary spherocytosis
chronic extravascular haemolysis
46
what does the defect in hereditary spherocytosis affect
RBC membrane flexibility
47
what type of haemolysis is seen in malaria
intravascular haemolysis
48
what is seen on blood film of HbH (severe alpha thalassaemia)
inclusion bodies- golf ball appearance
49
what are Heinz bodies
red cell inclusions made of denatured Hb that is normally removed by the spleen
50
what condition are Heinz bodies seen in
G6PD deficiency
51
splenomegaly, intermittent mild jaundice + gall stones | most likely diagnosis?
hereditary spherocytosis
52
Long standing splenomegaly, no spherocytes on blood film | most likely diagnosis?
Hb Koln disease
53
Child with anaemia, intermittent jaundice when unwell, asian ethnicity most likely diagnosis?
HbH alpha thalassaemia illness precipitates oxidative stress-- haemolysis + jaundice
54
what test detects methaemalbumin
schumm's test

Haematology Year 3 (21 decks)

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