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Haematology Y3 > Haemolytic anaemia (zero to finals) > Flashcards

Haemolytic anaemia (zero to finals) Flashcards

1
Q

What is haemolytic anaemia?

A

Involves the destruction of red blood cells (haemolysis), resulting in a low haemoglobin concentration (anaemia).

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2
Q

How do inherited conditions lead to chronic haemolytic anaemia?

A

Several inherited conditions cause the red blood cells to be more fragile and break down faster than normal, leading to chronic haemolytic anaemia.

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3
Q

What are some examples of inherited conditions that can lead to chronic haemolytic anaemia?

A

Hereditary spherocytosis

Hereditary elliptocytosis

Thalassaemia

Sickle cell anaemia

G6PD deficiency

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4
Q

What are several acquired conditions that lead to the destruction of the red blood cells?

A

Autoimmune haemolytic anaemia

Alloimmune haemolytic anaemia (e.g., transfusions reactions and haemolytic disease of newborn)

Paroxysmal nocturnal haemoglobinuria

Microangiopathic haemolytic anaemia

Prosthetic valve-related haemolysis

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5
Q

What are the main 3 features that can occur as a result of red cell destruction?

A

Anaemia

Splenomegaly (the spleen becomes filled with destroyed red blood cells)

Jaundice (bilirubin is released during the destruction of red blood cells)

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6
Q

Investigations for haemolytic anaemia?

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 (not in other types)

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7
Q

What is hereditary spherocytosis?

A

A common inherited haemolytic anaemia.

It causes fragile, sphere-shaped red blood cells that easily break down when passing through the spleen.

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8
Q

Inheritance pattern for hereditary spherocytosis?

A

Autosomal dominant

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9
Q

What is a notable feature of hereditary spherocytosis?

A

A notable feature is aplastic crisis in the presence of the parvovirus.

There is likely to be a positive family history.

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10
Q

What are key findings of hereditary spherocytosis?

A

Raised mean corpuscular haemoglobin concentration (MCHC) on a full blood count

Raised reticulocyte count due to rapid turnover of red blood cells

Spherocytes on a blood film

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11
Q

What is treatment of hereditary spherocytosis?

A

Treatment is with folate supplementation, blood transfusions when required and splenectomy.

Gallbladder removal (cholecystectomy) may be required if gallstones are a problem.

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12
Q

What is hereditary elliptocytosis?

A

Hereditary elliptocytosis is similar to hereditary spherocytosis except that the red blood cells are ellipse-shaped.

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13
Q

What is the inheritance pattern for hereditary elliptocytosis?

A

Autosomal dominant - same as hereditary spherocytosis.

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14
Q

What is the cause of G6PD deficiency?

A

Caused by a defect in the gene coding for glucose-6-phosphate dehydrogenase (G6PD), an enzyme responsible for protecting the cells from oxidative damage.

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15
Q

What is the inheritance pattern for G6PD deficiency?

A

X-linked recessive (where males are more often affected and females are carriers).

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16
Q

G6PD deficiency results in acute episodes of haemolytic anaemia, what are the triggers for this?

A

Infections, drugs or fava beans.

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17
Q

What are some key medication triggers for G6PD deficiency?

A

Ciprofloxacin, sulfonylureas (e.g., gliclazide) and sulfasalazine.

Another example is nitrofurantoin

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18
Q

Clinical features of G6PD deficiency?

A

G6PD deficiency presents with jaundice (often in the neonatal period), gallstones, anaemia, splenomegaly and Heinz bodies on a blood film.

19
Q

What are Heinz bodies on blood film?

A

A rounded structure protruding from the margin of an erythrocyte or as a small somewhat refractile spot within the cell.

Essentially it’s a small area that sticks out of the edge of the red blood cell.

20
Q

How is G6PD deficiency diagnosed?

A

Diagnosis can be made by doing a G6PD enzyme assay.

21
Q

What is the typical presentation of G6PD deficiency?

A

G6PD deficiency relates to triggers.

Typical presentation is a male patient that turns jaundiced and becomes anaemic after eating fava beans (broad beans), developing an infection or taking antimalarials.

22
Q

What is autoimmune haemolytic anaemia (AIHA)?

A

Occurs when antibodies are created against the patient’s red blood cells.

These antibodies lead to red blood cell destruction (haemolysis).

23
Q

What are the 2 types of AIHA?

A

There are two types, warm and cold, based on the temperature at which the auto-antibodies destroy red blood cells.

24
Q

Cold AIHA is more common than warm AIHA. True/false?

A

False

Warm AIHA is more common than cold

25
Q

What is warm AIHA?

A

Warm autoimmune haemolytic anaemia is the more common type. Haemolysis occurs at normal or above-normal temperatures.

It is usually idiopathic, meaning that it arises without a clear cause.

26
Q

What is cold AIHA?

A

Cold-reactive autoimmune haemolytic anaemia is also called cold agglutinin disease.

At lower temperatures (e.g., less than 10ºC), the antibodies attach to the red blood cells and cause them to clump together, called agglutination.

The immune system is activated, and the red blood cells are destroyed.

27
Q

What can cold AIHA occur secondary to?

A

Cold AIHA can be secondary to lymphoma, leukaemia, systemic lupus erythematosus and infections (e.g., mycoplasma, EBV, CMV and HIV).

28
Q

What is the management of AIHA?

A

Involves:

  • Blood transfusions
  • Prednisolone
  • Rituximab (a monoclonal antibody against B cells)
  • Splenectomy
29
Q

What is alloimmune haemolytic anaemia?

A

Alloimmune haemolytic anaemia occurs due to foreign red blood cells or foreign antibodies.

30
Q

What are the 2 scenarios where alloimmune haemolytic anaemia would happen?

A

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

31
Q

When do haemolytic transfusion reactions occur?

A

Haemolytic transfusion reactions occur when red blood cells are transfused into the patient.

The immune system produces antibodies against antigens on the foreign red blood cells.

An immune response leads to the destruction of those foreign red blood cells.

32
Q

When does haemolytic disease of the newborn occur?

A

Haemolytic disease of the newborn occurs when maternal antibodies cross the placenta from the mother to the foetus.

These maternal antibodies target antigens on the red blood cells of the foetus.

33
Q

What is the underlying pathology of haemolytic disease of the newborn?

A

These maternal antibodies destroy the neonate’s red blood cells. It occurs when the fetus is rhesus D positive (with rhesus D antigens on their red blood cells), and the mother is rhesus D negative (with no rhesus D antigens on her red blood cells).

34
Q

What is paroxysmal nocturnal haemoglobinuria caused by?

A

Paroxysmal nocturnal haemoglobinuria is caused by a specific genetic mutation in the haematopoietic stem cells in the bone marrow.

This mutation occurs during the patient’s lifetime (as opposed to being an inherited genetic condition).

35
Q

What are the characteristic presenting features of paroxysmal nocturnal haemoglobinuria?

A

The characteristic presenting symptom is red urine in the morning, which contains haemoglobin and haemosiderin.

Other presenting features are anaemia, thrombosis (e.g., DVT, PE and hepatic vein thrombosis) and smooth muscle dystonia (e.g., oesophageal spasm and erectile dysfunction).

36
Q

What is management of paroxysmal nocturnal haemoglobinuria?

A

Management is with eculizumab or bone marrow transplantation.

Eculizumab is a monoclonal antibody that targets complement component 5 (C5).

Bone marrow transplantation can be curative.

37
Q

What is microangiopathic haemolytic anaemia (MAHA)?

A

Microangiopathic haemolytic anaemia (MAHA) involves the destruction of red blood cells as they travel through the circulation.

38
Q

Cause of MAHA and how does it lead to haemolysis?

A

This is most often caused by abnormal activation of the clotting system, with blood clots (thrombi) partially obstructing the small blood vessels, referred to as thrombotic microangiopathy.

These obstructions churn the red blood cells, causing haemolysis (rupture).

39
Q

What conditions do MAHA usually occur secondary to?

A

Haemolytic uraemic syndrome (HUS)

Disseminated intravascular coagulation (DIC)

Thrombotic thrombocytopenic purpura (TTP)

Systemic lupus erythematosus (SLE)
Cancer

40
Q

What is a key finding on the blood film for MAHA?

A

Schistocytes are a key finding on the blood film in patients with microangiopathic haemolytic anaemia.

41
Q

What can haemolytic anaemia be a key complication of?

A

Haemolytic anaemia is a a key complication of prosthetic heart valves.

It occurs in both bioprosthetic and metallic valve replacement, although it varies depending on the type.

42
Q

How is haemolytic anaemia caused by heart valve replacement?

A

Caused by turbulence flow around the valve and the shearing of the red blood cells. The valve churns up the cells, and they break down.

43
Q

What is involved in the management of haemolytic anaemia as a result of prosthetic heart valves?

A

Monitoring

Oral iron and folic acid supplementation

Blood transfusions if severe

Revision surgery may be required in severe cases

Haematology Y3 (39 decks)

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