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Haematology > Haemolysis > Flashcards

Haemolysis Flashcards

1
Q

haemolysis

A

red cell destruction
shortened red cell survival

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2
Q

why are red cells susceptible to damage

A

no nucleus, mitochondria etc

rely on biconcave shape so any problem with shape they are gonna struggle

cant generate new proteins once in circulation

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3
Q

compensated haemolysis

A

increased red cell destruction compensated by increased red cell production

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4
Q

haemolytic anaemia

A

increased rate of rred cell destruction exceeding bone marrow capacity for red cell production

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5
Q

consequences of haemolysis

A

Erythroid hyperplasia

Reticulocytosis

Excess red cell breakdown products eg billirubin

clinical features differ by aetiology and site of red cell breakdown

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6
Q

can you directly measure red cell survival

A

no it relies on detecting the consequences of haemolysis and then investigating the cause

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7
Q

bone marrow response to haemolysis

A

reticulocytosis

erythroid hyperplasia

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8
Q

are reticulocytes nucleated cells

A

no

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9
Q

what is erythroid hyperplasia

A

increase in red cell precursors- erythyroids in bone marrow

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10
Q

most commonly used stain for reticulocytes

A

methylene blue as it stains the residual rna making them appear dark blue granular cells

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11
Q

diagnosis of haemolysis criteria

A

Evidence of increase red cell production
AND
Evidence of increased red cell breakdown products

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12
Q

products of red cell destruction

A

bilirubin

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13
Q

what happens if red cell destruction is outside the macrophage

A

free hb circulates and needs removed

can clog the kidneys and emerge in urine - haemoglobunuria

binds to albumin - methaemalbuminaemia

binds to haptoglobin and removed

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14
Q

how do classify where haemolysis is occuring

A

Pathophysiological approach – by site in the red cell where the problem is occurring

A practical approach by site in the body where the haemolysis is occurring

Extravascular (ie in the macrophage as normal)

Intravascular (ie in the circulation – not normal so generates a short differential list)

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15
Q

extravascular red cell destruction

A

breakdown outside blood vessels particularly in spleen, bone marrow and liver

more common

macrophages eat up aged or damaged cells

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16
Q

what does extravascular haemolysis release

A

protoporphyrin which can then be broken down into bilirubin

normal products but in excess

17
Q

intravascular red cell destruction

A

red cells destroyed in circulation spilling their contents immediately

not normal

18
Q

reactions occuring in intravascular haemolysis

A

Haemoglobinaemia (free Hb in circulation)

Methaemalbuminaemia (Hb mopped up by albumin)

Haemoglobinuria: pink urine, turns black on standing

Haemosiderinuria (iron taken up by tubular cells and converted to haemosiderin the cells are later shed into urine)

19
Q

intravascular causes

A

ABO incompatible blood transfusion

Mechanical RBC trauma (March haemoglobinuria,
DIC, HUS/TTP, valve disease)

G6PD deficiency if severe

Rarer still PNH,PCH, severe malaria, toxins
eg clostridia, some snake venoms

20
Q

extravascular causes

A

essentially all other causes of haemolysis

21
Q

investigations to confirm haemolytic state

A

FBC (+ BLOOD FILM) identify causes
Reticulocyte count (raised)
Serum unconjugated bilirubin (raised)
Serum haptoglobins (bind free Hb so will be low as consumed in haemolysis)
Urinary urobilinogen (raised)
Lactate dehydrogenase (raised) – non specific

22
Q

alloimmune haemolysis

A

make an antibody to someone elses red cells that have been given to you or an antibody is given to you that attacks your cells

23
Q

direct coombs test/ direct antiglobulin test use

A

lab test used to detect antibodies or complement proteins that are bound to the surface of red blood cells

24
Q

uses of dat

A

haemolytic anaemia, autoimmune disorders and haemolytic disease of new born

25
Q

igm assoc haemolysis

A

involved in cold agglutination disease

igm antibodies bind to red blood cells at lower temps <30

cause clumping of red blood cells

26
Q

abnormal situations resulting in premature destruction

A

toxins - chlosridia

snake venoms

rupture of infected red cells by malaria parasites

27
Q

hereditary spherocytosis

A

most common cause of hemolytic anaemia
caused by mutations in genes that code for proteins involved in the red blood cell membrane

These defects lead to the formation of abnormally shaped red blood cells (spherocytes) that are less flexible and more prone to breaking when passing through the spleen.

Symptoms include anemia, jaundice, and splenomegaly.

28
Q

what is a spherocyte

A

abnormally shaped red blood cell that is spherical in shape rather than the typical biconcave disk shape.

29
Q

causes of spherocytes

A

Hereditary Spherocytosis
Warm (IgG) haemolytic anaemia
Delayed transfusion reaction
Haemolytic disease of the newborn
Zieve’s Syndrome
Drug induced haemolysis
Fresh water drowning (but by a hypotonic effect)

30
Q

g6pd deficiency

A

geentic disorder affecting red blooc cells leading to haemolysis

x linked- male problem usually

31
Q

what does g69pd enzyme play a role in

A

protecting red blood cells from oxidative stress

32
Q

what is sickle cell disease

A

abnormal haemoglobin structure

caused by a point mutatoin in beta chain

result in shortened red cell production

33
Q

warm igg assoc haemolysis

A

igg antibodies bind to red blood cells at normal body temp leading to their premature destrcution

34
Q

pathophysiology of igg automminune hemolytic anemia

A

igg autoantibodies coat rbcs

recognised by macrpohages in the spleen by fc receptors

extravascular hemolysis occurs

partially phagocytosed leading to formation of spherocytes

35
Q
A

Haematology (21 decks)

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