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Haematology > Haemolysis > Flashcards

Haemolysis Flashcards

1
Q

what is haemolysis

A

premature red cell destruction

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2
Q

why are red blood cells susceptible to damage

A

biconcave shape
limited metabolic reserve
can’t generate new proteins once in circulation

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3
Q

what is compensated haemolytic

A

increased red cell destruction compensated by increased red cell production i.e. Hb maintained

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4
Q

what is hameolytic anaemia

A

decompensated haemolysis

increased rate of red cell destruction exceeding bone marrow capacity for red cell production i.e. Hb falls

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5
Q

consequence of haemolysi

A

erythroid hyperplasia - increased bone marrow RC production

excess RC breakdown products e.g. billirubin

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6
Q

what is the bone marrow response to haemolytic

A

reticulocytosis

erythroid hyperplasia

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7
Q

reticulocytosis other causes

A

bleeding

iron therapy in iron defic anaemia

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8
Q

stain for reticulocytes

A

supra vital stain staining ribosomal RNA

new methylene blue

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9
Q

way of counting reticulocytes

A

automated reticulocyte counting

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10
Q

classification of haemolytic

A

extravascular - taken up by reticuloendothelial system (spleen and liver predom)

intravascular - red cells destroyed within the circulation

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11
Q

extravascular red cell destruction

A

commoner
hyperplasia at site of destruction
release of protoporphyrin - unconjugated bilirubin - jaundice/gall stones. urobilinogenuria

normal products

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12
Q

intravascular red cell destruction

A

red cells destroyed in circulation spilling their contents

haemaglobinaemia
methaemalbuminaemia
haemaglobinuria: pink urine turns black on standing
haemosiderinuria

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13
Q

what type of haemolysis can be life threatening

A

intravascular

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14
Q

causes of intravascular

A

ABO incompatible blood transfusion
G6PD defic
severe falciparum malaria (blackwater fever)

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15
Q

causes of extravascular

A

all other causes of haemolysis

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16
Q

ix confirm haemolytic state

A 
confirm haemolytic state
FBC plus film
reticulocyte count
serum unconj bili
serum haptoglobin
urinary bili
17
Q

ix identify cause

A

history and exam
blood film: membrane damage (spherocytes), mechanical damage (red cell fragments), oxidative damage (heinz bodies), HbS (sickle cells)

18
Q

site of red cell defect classification

A

premature destruction of normal red cells (immune or mechanical)

abnormal cell membrane

abnormal red cell metabolism

abnormal Hb

19
Q

immune haemolysis

A

AI haemolysis

Alloimmune haemolysis

20
Q

AIH types

A 
warm (IgG):
idiopathic. commonest 
AI disorders (SLE)
lymphocytic disorders 
drugs (penicillin etc)
infections

cold (IgM):
idiopathic
infection (EBV, mycoplasma)
lymphoproliferative disorders

21
Q

what is the direct coombs test

A

identifies AB (and complement) bound to own red cells

22
Q

Alloimmune haemolysis

A 
immune response (AB produced):
haemolytic transfusion reaction - immediate IgM predom intravas or delayed IgG extra

passive transfer of antibody:
haemolytic disease of the newborn - RH D, ABO incompatibility, anti Kell

23
Q

mechanical red cell destruction

A 
DIC
haemolytic uraemic synd (E Coli 0157)
TTP
leaking heart valve
infection e.g. malaria
24
Q

mechanical valve related

A

microangiopathic haemolytic anaemia MAHA

25
Q

burns related haemolysis

A

microspherocytes
red cell are sheared as they pass through the damages capillaries

severe burns

26
Q

membrane defects acquired

A

liver disease - zieves synd
Vit E defic
Paroxysmal nocturnal Haemoglobinuria

27
Q

Zieves syndrome

A

haemolysis
alcoholic liver dx
hyperlipidaemia

anaemia, polychromatic macrocytes, irregularly contracted cells

28
Q

paroxysmal nocturnal Hb

A

film
ham’s test
marrow

acquired molecular defect - rare

29
Q

membran defects congenital

A

hereditary spherocytosis

30
Q

abnormal red cell metabolism congenital

A

GP6D defic - failure to cope with oxidant stress

31
Q

what does dapsone cause in px w Gp6D defic

features

A

haemolytic anaemia

keratocyte and irregularly contracted cell
Heinz bodies

32
Q

abnormal Hb congenital

A

Sickle cell dx

Haematology (26 decks)

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