haemolytic anaemia Flashcards

1
Q

what are clinical features of haemolytic anaemia?

A
  • Anaemia due to the reduction in circulating red blood cells
  • Splenomegaly as the spleen becomes filled with destroyed red blood cells
  • Jaundice as bilirubin is released during the destruction of red blood cells
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2
Q

what are inherited causes of haemolytic anaemia?

A

Hereditary Spherocytosis
Hereditary Elliptocytosis

Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency

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3
Q

what the causes of acquired haemolytic anaemia?

A
  • Autoimmune haemolytic anaemia
  • Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
  • Paroxysmal nocturnal haemoglobinuria
  • Microangiopathic haemolytic anaemia
  • Prosthetic valve related haemolysis
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4
Q

what type of anaemia will haemolytic anaemia cause?

A

normocytic anaemia

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5
Q

which investigations would you like to do when suspecting haemolytic anaemia and what will they show?

A
  • Full blood count shows a normocytic anaemia
  • Blood film shows schistocytes (fragments of red blood cells)
  • Direct Coombs test is positive in autoimmune haemolytic anaemia
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6
Q

what is the inheritance pattern for hereditary spherocytosis?

A

autosomal dominant, causing sphere shaped blood cells which are fragile and easily break down when passing through the spleen

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7
Q

what happens to reticulocytes in hereditary spherocytosis?

A

reticulocytes go up as there is a rapid turn over of rbc

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8
Q

which viral infection mmay cause aplastic crisis in someone with HS?

A

parvovirus

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9
Q

how can hereditary spherocytosis present?

A
  • jaundice
  • gallstones
  • splenomegaly
  • aplastic crisis with parvovirus
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10
Q

what is the management of hereditary spherocytosis?

A
  • folate supplementation
  • spplenectomy
  • cholecystectomy if there is a gallstone issue
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11
Q

how does hereditary ellipocytosis differ from HS?

A

RBC are ellipse shaped, but otherwise present and ar managed the same
also inherited in an autosomal fashion

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12
Q

what is the inheritence pattern for g6pd deficiency?

A

x linked recessive- only in males

more common in mediteraenean and african population

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13
Q

what is seen on the blood film of g6pd deficiency?

A

heinze bodies

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14
Q

what are the triggers for g6pd deficiency?

A
  • infections
  • medicatons
  • broad beans

causing gallstones, anaemia, splenomegaly and heinze bpodies

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15
Q

what are the 2 types of autoimmune haemolytic anaemia?

A

warm type: more common, haemolysis occurs at normal or above normal temps, and the cause is idiopathic

cold: happens at lower temperatures <10degrees . antibodies against rbc cause them to agglutinate, this results in destruction of RBC by the immune system. cold type haemolytic anaemia is usually secondary to…

  • lymphoma
  • leukaemia
  • SLE
  • EBV
  • mycoplasma
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16
Q

what is the management of autoimmune haemolytic anaemia?

A
  • Blood transfusions
  • Prednisolone (steroids)
  • Rituximab (a monoclonal antibody against B cells)
  • Splenectomy
17
Q

what is paroxysmal nocturnal haemaglobinuria?

A

mutation occurs in haematopoetic stem cells in the bone marrow during a patient’s lifetime.

this results in loss of protein on the surface of RBC which usually inhibit the complement cascade

activation of the complement cascade in the surface of rbc leads to destruction of RBC

18
Q

what is the presentation of someone with paroxysmal nocturnal haematuria?

A
  • red urine in the morning containing haemoglobin and haemosiderin

patient is also predisposed to thrombosis and smooth muscle dystonia-> oesophageal spasm and erectile dysfunction

19
Q

what is the management of paroxysmal nocturnal haematuria?

A
  • monoclonal antibody targeting complement- eculizumab

- bone marrow transplantation-> can be curative

20
Q

what are the causes of microangiopathic haemolytic anaemia?

A

Microangiopathic haemolytic anaemia (MAHA) is where the small blood vessels have structural abnormalities that cause haemolysis of the blood cells travelling through them

This is usually secondary to an underlying condition:

Haemolytic Uraemic Syndrome (HUS)
Disseminated Intravascular Coagulation (DIC)
Thrombotic Thrombocytopenia Purpura (TTP)
Systemic Lupus Erythematosus (SLE)
Cancer

21
Q

why can bioprosthetic and metalic valves cause haemolytic anaemia?

A

It is caused by turbulence around the valve and collision of red blood cells with the implanted valve

22
Q

what is the management of haemolytic anaemia caused by prosthetic valves?

A

Monitoring
Oral iron
Blood transfusion if severe
Revision surgery may be required in severe cases

23
Q

what are examples of alloimmune haemolytic anaemia?

A

Alloimmune haemolytic anaemia occurs where an there is either foreign red blood cells circulating in the patients blood causing an immune reaction that destroys those red blood cells

or there is a foreign antibody circulating in their blood that acts against their own red blood cells and causes haemolysis.

The two scenarios where this occurs are transfusion reactions and haemolytic disease of the newborn.

blood transfusion reaction: body produces antibodies against a foreign RBC

haemolytic disease of newborn: maternal antibodies cross the placenta and attack baby