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Haematology > Haemolysis > Flashcards

Haemolysis Flashcards

1
Q

What is the definition of haemolysis?

A

premature red cell destruction

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2
Q

Why are red cells particularly susceptible to damage?

A

need biconcave shape to transit circulation; limited metabolic reserve and rely on glycloysis; can’t geenrate new proteins in circulation

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3
Q

What is compensated haemolysis?

A

increased red cell destruction compensated by increased red cell production

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4
Q

What is haemolytic anaemia?

A

decompensated haemolysis

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5
Q

What are the consequences of haemolysis?

A

erythroid hyperplasia; excess red cell breakdown products

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6
Q

What is the bone marrow response to haemolysis?

A

reticulocytosis; erythroid hyperplasia

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7
Q

When else may reticulocytosis be seen?

A

response to bleeding; iron therapy in IDA

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8
Q

how is automated reticulocyte counting carried otu?

A

ribosomal RNA is albelled with fluorochrome and fluorescent cells are counted

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9
Q

What is the difference between extravascular and intravascular haemolysis?

A

extravascular- taken up by reticuloendothelial system (spleen and liver); intravascular- RBCs destroyed within the ciruclation

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10
Q

Is intra or extravascular haemolysis more common?

A

extravascular

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11
Q

What occurs with extravascular red cell destruction?

A

hyperplasia at site of destruction; release of protoporphyrin (unconjugated bilirubinaemia and urobilinogenuria

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12
Q

What is seen with intravascular red cell destruction?

A

haemoglobinaemia; methaemalbuminaemia; haemoglobinuria; haemosiderinuria

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13
Q

What is seen with haemoglobinuria?

A

pink urine; turns black on standing

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14
Q

What is the difference between the products of intravascualr and extravascular haemolysis?

A

intra- abnormal products whereas extravascular is normal products in excess

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15
Q

What are the causes of intravascular haemolysis?

A

ABO incompatibility; G6PD deficiency; severe flaciparum malaria (blackwater fever); PNH; PCH

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16
Q

What is seen on blood film with membrane damage?

A

spherocytes

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17
Q

Waht is seen on blood film with mechanical damage?

A

red cell fragmnets

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18
Q

What is seen on blood film with oxidative damage?

A

Heinz bodies

19
Q

How else can haemolysis be classified?

A

by red cell defect: 1- premature destruction of normal RBCs; abnormal cell memrbane; abnormal red cell maetabolism; abnormal Hb

20
Q

What are the causes of immun mediated haemolysis?

A

autoimmune haemolysis; alloimmune haemolysis

21
Q

What are the 2 types of autoimmune haemolysis?

A

warm and cold

22
Q

Waht antibodies are seen with warm AI haemolysis?

A

IgG

23
Q

What antibody is seen with cold AI haemolysis?

A

IgM

24
Q

What are the causes of warm AI haemolysis?

A

idiopathic; AI (eg SLE); lymphoproliferative disorders (CLL); drugs (penicillins); infections

25
Q

What are the causes of cold AI haemolysis?

A

idiopathic; infections (EBV, mycoplasma); lymphoproliferative disorders

26
Q

What is hte purpose of direct commbs test?

A

identifies antibody and complement cound to own red cells

27
Q

What is used in a direct coombs test?

A

patients RBCs (coated with IgG and complement)and mouse anti-human IgG

28
Q

What are hte causes of alloimmune haemolysis?

A

haemolytic transfusion reaction; haemolytic disease of the newborn

29
Q

What is the difference between the antibodies and haemolysis seen with haemolytic transfusion reactions?

A

immediate- IgM and intravascular; delayed- IgG and extravascular

30
Q

What are the causes of mechanical red cell destruction?

A

DIC; haemolytic uraemic syndrome (E.coli O157); TTP; leaking heart vlave; infection eg malaria

31
Q

what is microangiopathic haemolytic anaemia?

A

red cell fragmentation as a result of mechnical damage from damaged endothelial cells

32
Q

What is seen on blood film with burns related haemolysis?

A

microspherocytes

33
Q

What causes burns realted haemolysis?

A

red cells are sheared as they pass through damaged capillaries- only severe burns

34
Q

What are the causes of acquired membrane defects?

A

liver disease e.g Zieve’s syndrome; vitamin E deficiency; paroxysmal nocturnal haemoglobinuria

35
Q

What is the triad in Zieve’s syndrome?

A

haemolysis; alcoholic liver disease and hyperlipidaemia;

36
Q

What is seen on blood film with Zieve’s syndrome?

A

anaemia; polychromiatic macrocytes; irregularly contracted cells

37
Q

What is the characteristic sign assocaited with paroxysmal nocturnal haemoglobinuria?

A

red urine in the morning

38
Q

What causes PNH?

A

defective surface proteins on RBC causing a reactio n with the innate immune system

39
Q

what causes hereditary spherocytosis?

A

mutation in genes related to membrane proteins that allow cell to change shape

40
Q

What drugs can cause haemolysis due to disruption of metabolic pathways of normal RBCs?

A

dapsone; salazopyrin

41
Q

What is seen on blood film with dapsone therpay?

A

keratocyte and irregularly contracted cell

42
Q

What is HbH?

A

beta4

43
Q

Why do cells contained beta4 globin chains have reduced survival?

A

beta4 is toxic and prepitates when oxidative stress

44
Q

What are Heinz bodies?

A

red cell inclusions comprised of denatured Hb normally removed by spleen eg in G6PD deficiency

Haematology (29 decks)

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