Haemolysis Flashcards

1
Q

What is haemolysis?

A

premature red cell destruction

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2
Q

Why are RBCs susceptible to damage?

A

biconcave shape to transmit through circulation
limited metabolic reserve and rely on glucose metabolism for energy
they cannot generate new protiens once in circulation

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3
Q

What is compensated haemolysis?

A

increased red cell destruction that is compensated by increased red cell production

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4
Q

What is decompensated haemolysis?

A

increased red cell destruction exceeds bone marrow capacity for RBC production

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5
Q

What’re the consequences of haemolysis?

A

erythroid hyperplasia - increased bone marrow red cell production
excess red blood cell breakdown products eg bilirubin

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6
Q

What’re the signs of haemolysis?

A

reticulocytosis - have RNA so stain darker

erythroid hyperplasia

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7
Q

What two ways can haemolysis be classed?

A

extravascular

intravascular

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8
Q

What is extravascular haemolysis?

A

RBCs are taken up by the reticuloendothelial system - spleen and liver - and are destroyed - causes splenomegaly
causes the release of protoporphyrin = normal breakdown products in excess -> jaundice and gallstones

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9
Q

What is intravascular haemolysis?

A

RBCs are destroyed in the circulation, spilling their contents
this causes abnormal products - life threatening

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10
Q

What abnormal products are produced in intravascular haemolysis?

A

haemoglobinaemia - free Hb in circulation
methaemalbuminaemia
haemoglobinuria - pink urine that turns black on staining
haemosiderinuria

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11
Q

What causes intravascular haemolysis?

A

G6PD deficiency
ABO incompatibility
severe falciparum malaria

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12
Q

What are causes of premature haemolysis of normal RBCs?

A

autoimmune haemolysis

alloimmune haemolysis

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13
Q

What are the two types of autoimmune haemolysis?

A

Warm IgG

Cold IgG

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14
Q

What are causes of Warm IgG autoimmune haemolysis?

A
idiopathic
autoimmune disorders - SLE
lymphoproliferative disorders - CLL
drugs - penicillin
infections
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15
Q

What causes Cold IgG autoimmune haemolysis?

A

idiopathic
infections - EBV, mycoplasma
Lymphoproliferative disorders

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16
Q

What identifies autoimmune haemolysis?

A

Direct Coombs test - tests for antibodies already attached to RBCs

17
Q

What is alloimmune haemolysis?

A

antibodies develop to forgein red cells

18
Q

What are the two types of alloimmune haemolysis?

A

immune response

passive transfer of antibody

19
Q

What is immune response alloimmune haemolysis?

A

haemolytic transfusion reaction - can be delayed (IgG - extravascular) or acute (IgM - intravascular)

20
Q

What is passive transfer of antibody alloimmune haemolysis?

A

haemolytic disease of the newborn - ABO, RhD incompatibility

mechanical destruction - HUS, TTP, leaking heart valve (microangiopathic haemolytic anaemia), severe burns, malaria

21
Q

What aquired abnormal cell membrane defects lead to haemolysis?

A

liver disease -> cirrhosis -> zieves syndrome (alcoholics, polycromatic macrocytes, irregularly contracted cells)
vitamin E deficiency
paroxysmal nocturnal haemoglobinuria

22
Q

What congenital abnormal cell membrane defects lead to haemolysis?

A

Hereditary Spherocytosis

23
Q

What is hereditary spherocytosis?

A

autosomal dominant
reduced membrane deformability causing increased transit time through the spleen which means the oxidatiev environment in the spleen causes extravascular red cell destruction

24
Q

How does hereditary spherocytosis present?

A

gallstones
jaundice
splenomegaly

25
Q

How is hereditary spherocytosis treated?

A

splenectomy

26
Q

What congenital abnormalities to red cell metabolism can lead to haemolysis?

A

G6DP deficiency

27
Q

What congenital abnormalities to haemoglobin can lead to haemolysis?

A

sickle cell disease