Haemolysis Flashcards

1
Q

what is haemolysis?

A

premature destruction of red cells

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2
Q

why does bilirubin increase in haemolysis?

A

RBC breakdown breaks haemoglobin into its constituent parts

porphyrin ring breakdown eventually forms bilirubin

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3
Q

why are red cells susceptible to damage?

A

no nucleus - cant generate new proteins
limited metabolic reserve - rely on glycolysis
biconcave shape

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4
Q

what are Hb levels like in compensated haemolysis and why?

A

normal

haemolysis is compensated by inc RBC production

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5
Q

another term for decompensated haemolysis?

A

haemolytic anaemia

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6
Q

what is Hb like in haemolytic anaemia and why?

A

low

inc rate of haemolysis exceeds BM’s capacity for RBC production

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7
Q

what does the bone marrow do in response to haemolysis?

A

undergoes erythroid hyperplasia (inc BM red cell production) to produce more reticulocytes

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8
Q

what can you measure to look for haemolysis?

A

reticulocytes

breakdown products eg bilirubin

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9
Q

reticulocytosis is diagnostic of haemolysis T or F

A

F

present in bleeding, iron therapy etc

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10
Q

what cells are polychromatic and why?

A

reticulocytes

contain RNA

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11
Q

where is the main site of destruction in extravascular haemolysis

A

liver and spleen

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12
Q

where is the main site of destruction in intravascular haemolysis?

A

circulation

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13
Q

is extravascular or intravascular haemolysis more common?

A

extravascular

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14
Q

what happens to the organs at the site of destruction in extravascular haemolysis?

A

undergo hyperplasia

eg splenomegaly

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15
Q

what does the release of protoporphyrin in extravascular haemolysis cause?

A

unconj bilirubinaemia
jaundice
gallstones
urobilinogenuria

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16
Q

what haemolysis type is controlled and therefore does NOT have abnormal breakdown products?

A

extravascular haemolysis

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17
Q

pathophysiology of intravascular haemolysis?

A

red cells spill their contents into the bloodstream
free Hb in blood and urine
Hb breakdown products combine with other substances to form toxic products in blood and urine

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18
Q

colour of urine in haemoglobinuria?

A

pink, turns black on standing

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19
Q

what is the cause methaemalbuminaemia?

A

Hb breakdown product methaem forms a complex with albumin in the blood

20
Q

causes of intravascular haemolysis?

A

ABO incompatible blood transfusion
G6PD dfeficiency
malaria

21
Q

Ix haemolysis

A
FBC
blood film
reticulocyte count
serum unconj bilirubin
serum haptoglobins
urinary urobilinogen
22
Q

what should urine be checked for in haemolysis?

A

urobilinogen

23
Q

unconjugated or conjugated bilirubin increases in haemolysis?

A

unconjugated

24
Q

why is haptoglobin checked?

A

haptoglobin binds prehaemoglobin so if Hb is low from haemolysis this will also be low

25
Q

genetic causes of haemolysis

A

haemoglobinopathies
G6PD deficiency
thalassaemia
sickle cell (look for HbS)

26
Q

spherocytes indicate…

A

membrane damage

27
Q

heinz bodies indicate..

A

oxidative damage

28
Q

what factors can lead a red cell to haemolyse?

A

premature destruction
dysfunctional cell membrane
abnormal metabolism
abnormal Hb

29
Q

most common form of autoimmune haemolysis?

A

warm autoimmune haemolytic anaemia

30
Q

what antibody is involved in warm autoimmune HA?

A

IgG

31
Q

what antibody is involved in cold autoimmune HA?

A

IgM

32
Q

causes of warm autoimmune HA?

A
idiopathic
autoimmune disorder eg SLE
chronic lymphocytic leukaemia
drugs eg penicillins
infection
33
Q

causes of cold autoimmune HA?

A

idiopathic
infections eg EBV/mycoplasma
lymphoproliferative disorders

34
Q

what happens in a direct coombs test?

A

given antibodies to see if they bind to your own red cells (which causes agglutination)

35
Q

pathophysiology of autoimmune HA?

A

producing antibodies against your own RBC antigens

36
Q

causes of alloimmune haemolysis?

A

transfusion reaction

haemolytic disease of newborn due to rhesus/ABO cause

37
Q

if a transfusion reaction IMMEDIATELY causes haemolysis, what kind of haemolysis will it be and what antibody will cause it?

A

IgM

intravascular haemolysis

38
Q

if a transfusion reaction causes a DELAYED haemolysis, what kind of haemolysis will it be and what antibody will cause it?

A

IgG

extravascular

39
Q

causes of acquired haemolysis from MECHANICAL destruction?

A

coagulation disorder
HUS (e.coli 0157)
leaky heart valve
infections eg malaria

40
Q

microspherocytes are a sign of..

A

burns related haemolysis (sheared as they pass through damaged capillaries)

41
Q

name the triad of symptoms for zieve’s syndrome

A

haemolysis
alcoholic liver disease
hyperlipidaemia

42
Q

G6PD deficiency most often affects which members of the population?

A

MALES

43
Q

consequences of G6PD deficiency?

A

cant generate ATP

cant cope with oxidant stess

44
Q

bony deformities, chronic anaemia and erythroid hyperplasia…

A

beta thal major

45
Q

what is alloimmunity?

A

immune response to a foreign antigen

46
Q

parent had splenectomy, child has splenomegaly…

A

hereditary spherocytosis