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Haematology > Haem: Haemolytic Anaemias > Flashcards

Haem: Haemolytic Anaemias Flashcards

1
Q

What are the 2 types of haemolysis?

A

Intravascular - Within the circulation

Extravascular - Destruction by the reticular system/ spleen

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2
Q

What are the 2 ways haemolytic disorders can be classified?

A

Acquired

Inherited

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3
Q

What is the commonest cause of intravascular haemolytic anaemia?

A

Malaria - severe anaemia and haematurea

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4
Q

What are some other causes of intravascular haemolytic anaemia?

A

G6PD deficiency
Cold Ab haemolytic syndromes - IgM
MAHA - Damage to endothelium which causes damage to RBCs
Paroxysmal nocturnal haemaglobinuria - Genetic defect in GPI anchor

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5
Q

Give 2 examples of MAHA?

A

HUS in children whihc causes acute renal failure

TTP

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6
Q

What are the 3 types of disorders in hereditary haemolytic anaemia?

A

Membrane
RBC metabolism
Haemoglobin

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7
Q

What are some consequences of anaemia?

A

Increased RBC production and reticulocyte circulation
Increased folate demand
Susceptibility to Parvovirus B19 infection
Propensity to gallstones (cholelithiasis)
Iron overload
Osteoporosis

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8
Q

How does parvovirus affect anaemics?

A

Infect developing RBC and arrests their development.

Aplastic crisis - no RBC and no reticulocytes

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9
Q

Which gene is affected in Gilbert’s

A

UGT 1A1

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10
Q

How can you spot iron overload on histology

A

Blue stain with Prussian Blue/Perl’s stain

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11
Q

What are the clinical features of anaemia?

A

Pallor, jaundice, splenomegaly, pigmenturia, increased reticulocytes, polychromasia as RBCs take up eosinophilic and basophilic dyes giving a blue/purple appearance, hyperbilirubinaemia, Increased LDH, reduced haptoglobins, haemoglobinuria, haemosiderinurea

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12
Q

How does G6PD deficiency and PNH cause intravascular haemolysis?

A

Complement regulated proteins protect RBCs from complement in the plasma. These proteins are absent in these conditions.

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13
Q

What are the defects in RBC membranes in hereditary spherocytosis and elliptocytosis?

A

HS - Vertical interaction defects in the membranes (between bilayer and cytoskeleton0

HE - Horizontal interaction defects in the membrane

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14
Q

How is hereditary spherocytosis inherited?

A

Autosomal dominant

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15
Q

What are the features of hereditary spherocytosis?

A

Osmotic fragility - increased tendency to lyse
Lack area of central pallor
Biconcave shape
Hyperchromic cells
Smaller and more densely stained
Polychromatic cells - indicative of young cells
Reduced binding of the dye eosin-5-maleimide

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16
Q

What do you use to screen for hereditary spherocytosis?

A

Flow cytometry

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17
Q

How does hereditary elliptocytosis present?

A

Elliptical appearance
NO polychromasia
Blood count is normal

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18
Q

What is hereditary pyropoikilocytosis?

A

Homozygous state of hereditary elliptocytosis

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19
Q

How does hereditary pyropoikilocytosis present?

A

Fragmentation of RBCs

Poikilocytosis (different shapes)

20
Q

How is G6PD deficiency inherited?

A

X linked recessive

Seen mainly in hemizygous males and homozygous males

21
Q

What are the clinical presentations of G6PD deficiency?

A

Acute haemolytic anaemia
Neonatal jaundice
Impaired conjugation of bilirubin

22
Q

What can you see on a blood film for acute haemolytic anaemia?

A

Contracted cells
Nucleated blood cells
Bite cells - cytoplasm removed
Hemi ghosts - Hb is contracted to one side of the cell

23
Q

What can you see on the blood film of acute haemolytic anaemia in the steady state?

A

Heinz bodies - methylviolet stain

24
Q

What are some agents that can provoke G6PD deficiency?

A

Anti malarials - Primaquine

Abx - Sulphonamides, Ciprofloxacin, Nitrofurantoin

Dapsone
Vit K
Mothballs

Fava beans

25
Q

What is the most common defect in the glyocolytic pathway?

A

Pyruvate kinase deficiency (PK)

26
Q

What does the blood film of PK deficiency look like?

A

Echinocytes (Burr cells)
RBCs shrink in size and resemble spherocytes
Number of echinocytes increases after splenectomy

27
Q

What can lead poisoning lead to?

A

Pyrimidine 5’-nucleotidase deficiency

28
Q

What does the blood film of Pyrimidine 5’-nucleotidase deficiency show?

A

Basophllic stippling/ inclusion in RBCs

29
Q

Why would you do a direct antiglobulin test?

A

Detect immunoglobuline on RBCs - important in autoimmune haemolysis

30
Q

Why would you look for urinary haemosiderin / hb

A

Looking for intravascular haemolysis

LDH will be raised and haptoglobulin will be reduced

31
Q

Why would you look for osmotic fragility?

A

Membrane defect - Hereditary spherocytosis

32
Q

Difference between G6PD def and PK def?

A

G6PD - episodic acute haemolysis and often on exposure to oxidants
PK - Chronic haemolysis

33
Q

Why would you look for Hb separation A and F%

A

Hb disorders

34
Q

Why would you look for Heinz body stain?

A

Oxidative haemolysis

35
Q

Why would you look for Ham’s test/ flow cytometry of GPI linked proteins

A

PNH

Look at the sensitivity of RBCs to lysis via acidified serum - Ham’s test

36
Q

Why would you look for thick and thin blood film

A

Malaria

You also need immunological dipstick test for malarial antigens

37
Q

How do you manage haemolysis?

A

Folic acid
Avoid precipitators
RBC tranfuse/exchange
Immunisation against blood borne viruses

38
Q

What are the indications for splenectomy?

A 
PK deficiency 
Hereditary spherocytosis 
Severe elliptocytosis 
Pyropoikilocytosis
Thalassemia 
Immune haemolytic anaemia
39
Q

What is the biggest risk of splenectomy

A

Capsulated bacteria infections - Pneumococcus

40
Q

How can you mitigate pneumoccocal infections in splenectomy

A

Prophylactic penicillin and immunisation

41
Q

What are some specific indications for splenectomy

A 
Transfusion dependance
Growth delay
Hypersplenism 
Physical limitation of Hb
Between 3 and 10 years old
42
Q

What is Hb Hammersmith?

A

severe unstable Hb that produced Heinz body haemolytic anaemia

43
Q

What is the most important product of haemolytic anaemia?

A

Spherocytes

44
Q

Are spherocytes DAT +ve or -ve?

A

DAT -ve

45
Q

What is Waldenstrom’s macroglobulinaemia?

A

B cell clonal disorder
High levels of IgM
Feature s of hyperviscosity and vascular complications

46
Q

What is paroxysmal cold Hb

A

Caused by viral infections

Donath-Landsteiner antibodies

Haematology (10 decks)

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