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Haematology- Carol > 1.07 Haemolysis > Flashcards

1.07 Haemolysis Flashcards

1
Q

What reaction is catalysed by heme oxygenase?

What other molecules are needed?

A

The breakdown of haem to biliverdin
O2 and NADPH are needed
(produced include Fe2+, CO, H2O, NADP+)

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2
Q

What reaction is catalysed by biliverdin reductase?

A

The breakdown of biliverdin to bilirubin

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3
Q

Haemolysis

A

premature red cell destruction- shortened red ell survival

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4
Q

compensated haemolysis

A

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

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5
Q

Haemolytic anaemia

A

decompensated haemolysis
increased red cell destruction greater than bone marrow capacity for red cell production
hb falls

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6
Q

how is haemolysis and red cell survival detected?

A

assessing increased red cell production

detecting breakdown products

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7
Q

what is the normal bone marrow response to haemolysis?

A

reticulocytosis

erythroid hyperplasia

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8
Q

T/F: polychromasia is diagnositc of haemolysis?

A

False
polychromasia represents reticulocytes with ribosomal RNA
This could be due to a response to bleeding, iron therapy in iron deficiency, or haemolysis

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9
Q

what does new methylene blue stain for?

A

reticulocytes

this is a supravital stain for ribosomal RNA

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10
Q

how are reticulocytes automatically counted?

A

ribosomal RNA labelled with fluorochrome and those are counted

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11
Q

extravascular haemolysis

A

rbc taken up by reticuloendothelial system- spleen and liver +

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12
Q

intravascular haemolysis

A

red cells destroyed in circulation

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13
Q

What are the s/s of extravascular red cell destruction?

A

hyperplasia at site of destruction ( spleen +/- liver)
release of normal products in excess: protoporphyrin
-unconjugated bilirubinaemia–> jaundice, gallstones
-urobilinogenuria

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14
Q

What are the abnormal products found in intravascular haemolysis?

A

haemoglobinaemia
methaemalbuminaemia
haemoglobinuria- pink urine that turns black
haemosiderinuria

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15
Q

T/F: Intravascular haemolysis may be life threatening?

A

True

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16
Q

Intra or extravascular:

ABO incompatability

A

Intravascular

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17
Q

Intra or extravascular:

Glucose-6 phosphatase deficiency

A

intravascular

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18
Q

Intra or extravascular:

severe falciparum malaria- blackwater fever

A

intravascular

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19
Q

Intra or extravascular:

paroxysmal noctural haematuria

A

Intravascular

20
Q

what is haptoglobin?

A

protein in the blood that binds free haemoglobin
can help differentiate extravascular from intravascular haemolysis (if decreased, intravascular, unless excessive extravascular)
-not terribly discriminative

21
Q

What tests confirm haemolytic state?

A 
FBC
blood film 
retic count
serum unconjugated bilirubin
serum hpatoglobins
urinary urobilinogen
22
Q

spherocytes on blood film

A

membrane damage

23
Q

red cell fragments on blood film

A

mechanical damage

24
Q

heinz bodies on blood film

A

oxidative damage

25
Q

HbS on blood film

A

sickle cells

26
Q

Warm autoantibody

A

IgG

  • idiopathic
  • autoimmune- SLE
  • lymphoproliferative- CLL
  • drugs- penicillins, etc.
  • infection
27
Q

Cold autoantibody

A

IgM

  • idiopathic
  • infections-EBV, mycoplasma
  • lymphoproliferative
28
Q

What antibody is coated on RBC for detection on Direct Coombs Test?

A

IgG + complement on RBC
mouse anti-human IgG is added

agglutination indicates there is antibody and complement bound to the patient’s own red cells.

29
Q

immediate haemolytic transfusion reaction

A

IgM (ImMediate)

intravascular (couldn’t wait to make it to spleen/liver)

30
Q

Delayed haemolytic transfusion reaction

A

IgG (lonGer after)

Extravascular (was able to make it to spleen/liver)

31
Q

Passive transfer of antibody in haemolytic disease of newborn

  • RhD
  • ABO incompat
  • Anti-Kell, Duffy, etc.

is alloimmune or autoimmune?

A

Alloimmune haemolysis

32
Q

causes of mechanical red cell destruction

A 
DIC
HUS- E Coli 0157 
TTP 
leaking heart valve
infections eg. malaria
33
Q

microspherocytes

A

burns related haemolysis

34
Q

causes of acquired haemolysis - membrane defects

A

liver disease
vitamin E deficiency
paroxysmal nocturnal haemoglobinuria

35
Q

Liver disease- Zieve’s syndrom

A

an acute metabolic condition that can occur during withdrawal from prolonged alcohol abuse

haemolysis
alcoholic liver disease
hyperlipidaemia 
anaemia
polychromatic macrocyets
irregularly contracted cells
36
Q

how is paroxysmal nocturnal haemoglobinruia detected?

A

blood film
ham’s test- acid lysis
bone marrow

rare cause of haemolysis

37
Q

why do RBC need G6P?

A

to make antioxidant and ATP

and reduce glucose to lactate

38
Q

What drugs may sufficiency stress rbc and cause abnormal rbc metabolism?

A

dapsone

salazopyrin

39
Q

dapsone therapy

A

keratocyte

irregularly contracted cell

40
Q

haemoglobin S- sickle cell disease

A

abnormal haemoglobin polymerisation

shortened red cell survival

41
Q

beta thalassaemia major
clinical s/s
blood film
bone marrow

A

s/s: bony deformities

film: hypo/micro targets, nucleated RBCs, pappenheimer bodies, alpha chain ppts
marrow: erythroid hyperplasia

42
Q

what is the inheritance pattern of hereditary spherocytosis?

A

autosomal dominant

43
Q

what is the gene mutation in hereditary spherocytosis, the most common hereditary haemolytic anaemia?

A

spectrin gene

44
Q

Hb-Koln

A

unstable Hb variant

chronic haemolysis

45
Q

What is the number of abnormal Hb-a genes are required for HbH- alpha thalassaemia?

A

3 genes

if 4- Barts disease= hydrops fetalis, if 2 or 1 then normal Hb

46
Q

What is seen on the blood film of HbH disease?

A

alpha thalassaemia is seen as:
-HbH B4 containing golf balls
reticulocytes
B4 is toxic and ppts when there is oxidative stress eg. dapsone

47
Q

What are heinz bodies?

A

red cell inclusions
denated HB
these cells normally removed by spleen

causes:
G6PD
unstable Hb eg. HbH- alpha thalassaemia

Haematology- Carol (29 decks)

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