haemolytic anaemia

Question 1

what are clinical features of haemolytic anaemia?

Answer 1

• 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

Question 2

what are inherited causes of haemolytic anaemia?

Answer 2

Hereditary Spherocytosis
Hereditary Elliptocytosis

Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency

Question 3

what the causes of acquired haemolytic anaemia?

Answer 3

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

Question 4

what type of anaemia will haemolytic anaemia cause?

Answer 4

normocytic anaemia

Question 5

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

Answer 5

• 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

Question 6

what is the inheritance pattern for hereditary spherocytosis?

Answer 6

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

Question 7

what happens to reticulocytes in hereditary spherocytosis?

Answer 7

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

Question 8

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

Answer 8

parvovirus

Question 9

how can hereditary spherocytosis present?

Answer 9

• jaundice
• gallstones
• splenomegaly
• aplastic crisis with parvovirus

Question 10

what is the management of hereditary spherocytosis?

Answer 10

• folate supplementation
• spplenectomy
• cholecystectomy if there is a gallstone issue

Question 11

how does hereditary ellipocytosis differ from HS?

Answer 11

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

Question 12

what is the inheritence pattern for g6pd deficiency?

Answer 12

x linked recessive- only in males

more common in mediteraenean and african population

Question 13

what is seen on the blood film of g6pd deficiency?

Answer 13

heinze bodies

Question 14

what are the triggers for g6pd deficiency?

Answer 14

• infections
• medicatons
• broad beans

causing gallstones, anaemia, splenomegaly and heinze bpodies

Question 15

what are the 2 types of autoimmune haemolytic anaemia?

Answer 15

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

Question 16

what is the management of autoimmune haemolytic anaemia?

Answer 16

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

Question 17

what is paroxysmal nocturnal haemaglobinuria?

Answer 17

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

Question 18

what is the presentation of someone with paroxysmal nocturnal haematuria?

Answer 18

• red urine in the morning containing haemoglobin and haemosiderin

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

Question 19

what is the management of paroxysmal nocturnal haematuria?

Answer 19

• monoclonal antibody targeting complement- eculizumab

- bone marrow transplantation-> can be curative

Question 20

what are the causes of microangiopathic haemolytic anaemia?

Answer 20

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

Question 21

why can bioprosthetic and metalic valves cause haemolytic anaemia?

Answer 21

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

Question 22

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

Answer 22

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

Question 23

what are examples of alloimmune haemolytic anaemia?

Answer 23

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