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Haemotology > hemolysis > Flashcards

hemolysis Flashcards

1
Q

what is hemolysis?

A

premature red cell destruction

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2
Q

why are red cells particularly susceptible to damage? (3)

A

biconcave shape
limited metabolic reserve - no mitochondria
no nucleus (no new proteins)

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3
Q

what metabolism do red cells depend on?

A

glucose metabolism

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4
Q

what is compensated hemolysis?

A

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

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5
Q

what is most commonly seen?

A

haemolytic anaemia

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6
Q

what is hemolytic anaemia?

A

increased rate of red cell destruction exceeding bone marrow capacity for red cell production - Hb falls

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7
Q

what are the consequences of hemolysis? (2)

A

erythroid hyperplasia

excess red cell breakdown products eg. bilirubin

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8
Q

bone marrow response to haemolysis?

A

reticulocytosis

erythroid hyperplasia

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9
Q

what stains reticulocyte ribosomal RNA?

A

new methylene blue

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10
Q

what is extravascular hemolytic anaemia?

A

taken up by reticuloendothelial system (spleen and liver predominantly)

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11
Q

what is intravascular hemolytic anaemia?

A

red cells destroyed within the circulation

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12
Q

what is the common form of hemolytic anaemia?

A

extravascular

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13
Q

chronic extravascular hemolytic anaemia may present with?

A

splenomegaly +/- hepatomegaly

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14
Q

what type of. products are released in extravascular red cell destruction?

A

normal products in excess

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15
Q

what products are released in extravascular red cell destruction?

A

unconjugated bilirubinaemia

urobilinogenuria

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16
Q

what is the presentation of unconjugated bilirubinaemia?

A

jaundice and gall stones

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17
Q

what is intravascular red cell destruction?

A

red cells are destroyed in the circulation spilling their contents

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18
Q

what products are released in intravascular red cell destruction?

A

haemoglobinaemia
methaemalbuniaemia
haemolgobinuria
haemosiderinuria

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19
Q

what happens to hemoglobinuria?

A

pink urine turns black on oxidation

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20
Q

what type of products are released in intravascular red cell destruction?

A

abnormal products

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21
Q

which type of haemolysis is life threatening?

A

intravascular

22
Q

what conditions cause intravascular hemolysis? (4)

A

ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria (Blackwater Fever)
Rarer still PNH,PCH

23
Q

what is the most common red cell enzyme deficiency?

A

G6PD deficiency

24
Q

what conditions cause extravascular hemolysis?

A

essentially all other causes of hemolysis

25
Q

what investigations confirm hemolytic state? (5)

A 
FBC (+ BLOOD FILM)
Reticulocyte count
Serum unconjugated bilirubin
Serum haptoglobins
Urinary urobilinogen
26
Q

what excludes someone haemolysing?

A

FBC and blood film

27
Q

what do you see on the blood film if red cell membranes are damaged?

A

spherocytes

28
Q

what do you see on the blood film if there is oxidative damage?

A

Heinz bodies

29
Q

what do you see on the blood film If there is mechanical damage?

A

red cell fragments

30
Q

what is a specialist investigation for hemolysis?

A

direct coombs’ test and others

31
Q

what is the most common cause of premature destruction of normal red cells?

A

autoimmune, or mechanical

valve disease

32
Q

autoimmune hemolysis?

A

antibodies developed to own red cell

33
Q

what is alloimmune hemolysis?

A

antibodies developed to foreign red cells

34
Q

what are warm autoantibodies?

A

IgG

35
Q

what are cold autoantibodies?

A

IgM

36
Q

what is the commonest cause of warm autoimmune hemolysis?

A

idiopathic

37
Q

what are other warm autoimmune hemolysis?

A 
Idiopathic (commonest)
				Autoimmune disorders (SLE)					Lymphoproliferative disorders (CLL)			
Drugs	(penicillins, etc) - high doses given IM 					Infections
38
Q

what are causes of cold autoimmune hemolysis?

A

idiopathic
infections (EBV, mycoplasma)
Lymphoproliferative

39
Q

what is the commonest form of autoimmune hemolysis?

A

warm (IgG) hemolysis

40
Q

what is the purpose of direct coombs’ test?

A

identifies antibody (and complement) bound to own red cells

41
Q

alloimmune hemolysis can be?

A 
immune response (antibodies produced) 
passive transfer of antibody
42
Q

what is an example of alloimmune immune response hemolysis?

A

hemolyticc transfusion reaction

43
Q

how can you differentially if the HTR is predominantly intravascular or extravascular?

A

Immediate IgM - intravascular

delayed IgG - extravascular

44
Q

what are examples of passive transfer of antibody in alloimmune hamemolysiss?

A

hemolytic disease of the newborn

45
Q

what are causes of acquired haemolysis - mechanical red cell destruction?

A 
Disseminated intravascular coagulation
Haemolytic uraemic syndrome (eg E. coli O157)
TTP
Leaking heart valve
Infections e.g. Malaria
46
Q

what are examples of membrane defects?

A 
Liver Disease (Zieve’s Syndrome)
Vitamin E deficiency
Paroxysmal Nocturnal Haemoglobinuria
47
Q

what is a genetic cause of red cell membrane abnormalities?

A

hereditary spherocytosis

48
Q

people with normal levels of G6PD are also under

A

oxidative stress then they are on dapsone therapy

49
Q

what is an example of abnormal hemoglobin?

A

sickle cell disease

50
Q

what is affected in sickle cell disease>

A

physical properties of haemoglobin (abnormal polymerisation) resulting in shortened red cell survival

51
Q

where is the mutation in sickle cell disease?

A

caused by a point mutation in beta globin chain

52
Q

why isn’t the presence of spherocytes diagnostic in hereditary spherocytosis?

A

it can be seen in severe burns

immune hemolytic anaemias

Haemotology (7 decks)

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