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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
Q

subtypes of autoimmune haemolysis

A

warm (IgG)

cold (IgM)

26
Q

What antibody is involved in warm autoimmune haemolysis

A

IgG

27
Q

what antibody is involved in cold autoimmune haemolysis

A

IgM

28
Q

causes of warm autoimmune haemolysis

A 
Idiopathic (most common)
autoimmune disorders (SLE)
lymphoproliferative disorders (CLL) 
drugs (penicillin)
infections
29
Q

causes of cold autoimmune haemolysis

A

idiopathic
infections (EBV, Mycoplasma)
Lymphoproliferative disorders

30
Q

test for autoimmune haemolytic anaemia

A

direct Coomb’s test

31
Q

two forms of alloimmune haemolysis

A

immune response (antibody produced)

passive transfer of antibody

32
Q

what can cause an immune response leading to alloimmune haemolysis

A

Haemolytic transfusion reaction:
IgM : immediate (intravascular)
IgG: delayed (extravascular)

33
Q

what can cause a passive transfer of antibody leading to haemolysis

A

haemolytic disease of the new born

34
Q

causes of mechanical red cell destruction

A 
disseminated intravascular coagulation
haemolytic uraemia syndrome (e.g. caused by E.Coli O157)
TTP
leaking heart valve
malaria
35
Q

what is seen in a blood film if there has been mechanical red cell destruction

A

red cell fragmentation

36
Q

what is seen in a blood film if there has been burns related haemolysis

A

microspherocytes- small spherocytes predominate and RBCs are smaller and more globular than normal

37
Q

causes of acquired RBC membrane defects (rare)

A

Liver disease – Zieve’s syndrome
Vitamin E deficiency
Paroxysmal nocturnal haemoglobinuria

38
Q

what is Zieve’s syndrome

A

Haemolysis, alcoholic liver disease, hyperlipidaemia

39
Q

causes of congenital RBC membrane defect

A

hereditary spherocytosis

40
Q

genetic causes of abnormal red cell metabolism

A

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

failure to generate ATP: metabolic process fail

41
Q

what can cause stress to metabolic pathways in normal cells

A

dapsone or salazopyrin

42
Q

what does dapsone cause

A

bite cells a.ka. irregularly contracted cell

43
Q

what genetic disease can cause haemolytic anaemia by making abnormal haemoglobin

A

sickle cell disease

44
Q

what are pappenheimer bodies

what condition are they seen in

A

abnormal granules of iron found inside red blood cells

beta thalassaemia major

45
Q

what type of haemolysis is seen in hereditary spherocytosis

A

chronic extravascular haemolysis

46
Q

what does the defect in hereditary spherocytosis affect

A

RBC membrane flexibility

47
Q

what type of haemolysis is seen in malaria

A

intravascular haemolysis

48
Q

what is seen on blood film of HbH (severe alpha thalassaemia)

A

inclusion bodies- golf ball appearance

49
Q

what are Heinz bodies

A

red cell inclusions made of denatured Hb that is normally removed by the spleen

50
Q

what condition are Heinz bodies seen in

A

G6PD deficiency

51
Q

splenomegaly, intermittent mild jaundice + gall stones

most likely diagnosis?

A

hereditary spherocytosis

52
Q

Long standing splenomegaly, no spherocytes on blood film

most likely diagnosis?

A

Hb Koln disease

53
Q

Child with anaemia, intermittent jaundice when unwell, asian ethnicity
most likely diagnosis?

A

HbH
alpha thalassaemia
illness precipitates oxidative stress– haemolysis + jaundice

54
Q

what test detects methaemalbumin

A

schumm’s test

Haematology Year 3 (21 decks)

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