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Haematology > Haemolysis > Flashcards

Haemolysis Flashcards

1
Q

what is haemolysis

A

premature red ell destruction ie shortened red cell survival

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2
Q

what is compensated haemolysis

A

increased red cell destruction compensated by increased red cell production ie Hb maintained

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3
Q

what are the consequences of haemolysis

A 
erythroid hyperplasia (increae red cell production) 
excess bilirubin
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4
Q

what is the bone marrow response to haemolysis

A

reticulocytosis

erythroid hyperplasia

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5
Q

what do reticulocytes show on staining

A

supravital stain staining ribosomal RNA-new methylene blue

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6
Q

how are reticulocytes measured

A

automated reticulocyte counting

ribosomal RNA is labelled with a fluorochrome and fluorescent cells are counted

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7
Q

how can haemolysis be classified

A

extravascular and intravascular

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8
Q

what does extravascular mean

A

taken up by reticulendothelial system (spleen and liver)

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9
Q

what does intravascular mean

A

red cells destroyed within the circulation

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10
Q

in terms of products what does extravasucalr red cell destruction produce

A

normal products in excess

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11
Q

in terms of produces what does intravascular haemolysis produce

A

abnormal products

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12
Q

what does intravascular produce

A

haemoglobinaemia
methaemalbuminaemia
haemoglobinuria
haemosiderinuria

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13
Q

what does haemoglobinuria do

A

pink urine, turns black on standing

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14
Q

what does extravascular release

A

protoporphyrin

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15
Q

does extravascular cause a conjugated or unconjugated bilirubinaemia

A

unconjugated

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16
Q

what can intravascular be caused by

A

ABO incompatibile blood transfusion
G6PD deficiency
sever falciparum malaria-Blackwater fever

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17
Q

what investigations would you do to confirm the haemolytic state

A 
FBC (+ BLOOD Film)
reticulocyte count 
serum unconjugated bilirubin 
serum haptoglobins 
urinary urobilinogen
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18
Q

how else can it be classified-by site of red cell defect which are

A

premature destruction of normal red cells
abnormal cell membrane
abnormal red cell metabolism
abnormal haemoglobin

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19
Q

causes of haemolysis acquired

A

immune
autoimmune haemolysis
alloimmune haemolysis

20
Q

what are some of the causes of warm autoantibody

A

autoimmune disorders (SLE)
lympohoproliferative
drugs (penicillins), infections

21
Q

what immunoglobulin causes warm autoantibodies

A

IgG

22
Q

what immunoglobulin causes cold antibodies

A

IgM

23
Q

what causes cold autoimmune haemolysis

A

infections (EBV, mycoplasma) , lymphoproliferative disorders

24
Q

describe the direct Coomb’s test

A

patients RBC are coater with IgG and complement and added to mouse anti-human IgG to see if agglutination occurs

25
Q

what does Direct Coombs identify

A

antibody and complement bound to own red cells

26
Q

what is alloimmune haemolysis caused by

A

immune response to antibody produced or passive transfer of antibody

27
Q

causes of antibody produced alloimmune haemolysis

A

haemolytic transfusion reaction

28
Q

an immediated haemolytic transfusion reaction is intravascular or extravascular

A

intravascular IgM

29
Q

a delayed haemolytic transfusion reaction is caused by

A

delayed IgG predominantly extravascular

30
Q

when does passive transfer of antibody occur

A

in haemolytic disease of the newborn

31
Q

causes of acquired haemolysis

A

mechanical red cell destruction-DIC, HUS
TTP
leaking heart valve
infections eg malaria

32
Q

what does MAHA stand for and when does it occur

A

microangiopathic haemolytic anaemia (MAHA) result of mechanical damage

33
Q

what type of cells do you get in burns related haemolysis

A

microspherocytes

34
Q

why do you get microspherocytes in burns

A

red cells are sheard as they are passed through the damaged capillaries

35
Q

name some acquired membrane defects

A

these are all v rare-liver disease (Zieve’s syndrome), vitamin E deficiency, paroxysmal nocturnal hameoglobinuria

36
Q

what does Zieves disease look like

A

anaemia, polychromatic macrocytes, irregularly contracted cells

37
Q

what is Zieves syndrome

A

haemolysis, alcoholic liver disease, hyperlipidaemia)

38
Q

describe hereditary spherocytosis

A

reduced membrane deformity
increased transit time trough spleen
oxidant environment in spleen causes extravascular red cell destruction

39
Q

what can cause genetic abnormal red cell metabolism

A

failure to cope with oxidant stress (G6PD deficiency)

failure to generate ATP

40
Q

what drug can cause even normal cells to abnormally respond to oxidative stress

A

dapsone -which can be used to treat leprosy

41
Q

what is a genetic cause of abnormal haemoglobins

A

sickle cell disease HbS

42
Q

how does sickle cell affect haemoglobin

A

abnormal polymerisation which results in shortened red cell survivial

43
Q

what is sickle cell caused by

A

point mutation in B globin chain

44
Q

what signs do you have if you are sickle cell trait

A

none-asymptomatic

45
Q

Heinz bodies are seen in what type of anaemia

A

G6PD deficiency

Haematology (14 decks)

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