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Clinical lectures: Yr2 > Haemolytic Anaemia > Flashcards

Haemolytic Anaemia Flashcards

1
Q

What is haemolysis?

A

Erythrocyte breakdown before their normal 120-day lifespan

Haemolysis leads to a decrease in the number of red blood cells in circulation.

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2
Q

Define haemolytic anaemia

A

Form of anaemia that occurs when erythrocyte destruction happens at a faster rate than erythropoiesis

It is characterized by reduced erythrocyte production or increased breakdown.

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3
Q

What is erythropoietic hyperplasia?

A

Increased RBC production in the bone marrow at 6-8x normal rate

This occurs in response to increased destruction of erythrocytes.

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4
Q

List the laboratory findings associated with haemolytic anaemia

A
  • Elevated reticulocyte count (reticulocytosis)
  • Elevated lactate dehydrogenase
  • Low haptoglobin
  • Elevated unconjugated bilirubin
  • Blood film: polychromasia, nucleated erythrocytes

These findings help diagnose the condition.

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5
Q

What does elevated reticulocyte count indicate?

A

Increased RBC production in response to haemolysis

Reticulocytosis is a common finding in haemolytic anaemia.

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6
Q

What causes low haptoglobin levels in haemolytic anaemia?

A

Haptoglobin binds to free Hb released from dead/damaged erythrocytes and is used up faster than it can be produced by the liver

This is a key laboratory finding in diagnosing haemolytic anaemia.

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7
Q

What are common signs of haemolysis?

A
  • Dark urine
  • Jaundice (yellow sclera and skin)
  • Gallstones with RBC pigment
  • Anaemic signs (more common in acute haemolysis)

Dark urine is due to bilirubin conversion to urobilinogen.

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8
Q

What does a blood film show in haemolytic anaemia?

A

Normocytic or macrocytic erythrocytes due to raised reticulocyte count

Polychromasia and nucleated erythrocytes may also be observed.

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9
Q

What is paroxysmal nocturnal haemoglobinuria?

A

Acquired, rare form of intravascular haemolysis due to absence of glycosylphosphatidylinositol (GPI)

This absence leads to a lack of surface proteins that protect erythrocytes from complement-mediated lysis.

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10
Q

What are the symptoms of paroxysmal nocturnal haemoglobinuria?

A
  • High level of free Hb in blood
  • Dark urine
  • Increased risk of clot formation in unusual areas

These symptoms occur due to the breakdown of erythrocytes.

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11
Q

True or False: Gallstones with RBC pigment are more common in chronic haemolysis.

A

True

This is a result of persistent breakdown of red blood cells.

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12
Q

Fill in the blank: The protein produced in the liver that binds to free hemoglobin is called _______.

A

haptoglobin

Haptoglobin levels decrease in haemolytic conditions.

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13
Q

What are Haemaglobinopathies?

A

Inherited abnormalities of hemoglobin that can affect red blood cell function.

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14
Q

What are Membranopathies?

A

Inherited abnormalities characterized by erythrocytes that can’t maintain biconcave shape, making them more susceptible to damage and resulting in a shorter lifespan due to early macrophage removal in the spleen.

Examples include Hereditary Spherocytosis (HS), Hereditary Elliptocytosis (HE), and Hereditary Stomatocytosis.

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15
Q

How is Hereditary Spherocytosis (HS) treated?

A

Treated with folic acid supplementation and splenectomy.

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16
Q

What investigations are done for Membranopathies?

A

Investigations include family history, genetic testing (usually autosomal dominant), abnormal blood film, haemolysis screen, and specialist tests.

Tests show elevated bilirubin, elevated reticulocyte count, and elevated LDH, with a negative Coombe’s test indicating erythrocytes aren’t coated in antibody.

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17
Q

What are the clinical presentations of Membranopathies?

A

Clinical presentation includes prolonged neonatal jaundice, mild anemia at any age, fluctuating jaundice worse during infections, pigment gallstones, and aplastic crisis caused by parvovirus (B19).

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18
Q

What are Enzymopathies?

A

Disorders caused by enzyme deficiencies affecting red blood cells.

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19
Q

What is the most common Enzymopathy?

A

The most common is G6PD deficiency.

It is usually X-linked recessive.

20
Q

What is the epidemiology of G6PD deficiency?

A

Common in people with Mediterranean and African ancestry.

21
Q

What is the effect of G6PD deficiency on erythrocytes?

A

Erythrocytes can’t produce much NADPH for glutathione regeneration, making the RBC membrane unstable against oxidative stress caused by hydrogen peroxide.

22
Q

What typically triggers hemolysis episodes in G6PD deficiency?

A

Hemolysis episodes are usually triggered by oxidative stress caused by drugs, infections, mothballs, and fava beans.

23
Q

What are the extrinsic causes of haemolysis?

A

Extrinsic causes of haemolysis include antibody attack, specifically Autoimmune Haemolytic Anaemia (AIHA).

24
Q

What happens to erythrocytes in AIHA?

A

Erythrocytes will be coated by either IgM or IgG antibodies.

25
Q

What does a blood film show in IgM mediated AIHA?

A

Blood film shows agglutination.

26
Q

What does a blood film show in IgG mediated AIHA?

A

Blood film shows spherocytes.

27
Q

What is the main investigation for AIHA?

A

The main investigation is the Direct Coombe’s (antiglobulin) test.

28
Q

What indicates a positive Direct Coombe’s test?

A

Agglutination occurs when anti-human antibody is added to the patient sample of RBCs.

29
Q

What characterizes warm AIHA?

A

Warm AIHA occurs when antibodies react with RBCs at 37°C, usually involving IgG.

30
Q

What are common causes of warm AIHA?

A

Common causes include idiopathic reasons, autoimmune conditions, immune disorders like low-grade lymphoma or Chronic Lymphocytic Leukaemia (CLL), and medications like penicillin and methyldopa.

31
Q

What is the result of warm antibodies in AIHA?

A

Warm antibodies lead to extravascular haemolysis in the spleen and liver.

32
Q

What characterizes cold AIHA?

A

Cold AIHA occurs when antibodies react with RBCs below 37°C, usually involving IgM.

33
Q

What are common causes of cold AIHA?

A

Common causes include idiopathic reasons, low-grade lymphomas, and infections like mycoplasma pneumonia and EBV.

34
Q

What is Paroxysmal cold haemoglobinuria?

A

It is a rare form of cold AIHA where IgG antibody binds to erythrocytes in the cold but causes haemolysis in warm temperatures.

35
Q

What are the management strategies for AIHA?

A

Management includes treating the underlying cause, keeping the patient warm for cold AIHA, folic acid supplements, immunosuppressants, and splenectomy for most warm cases.

36
Q

What is the treatment for cold AIHA?

A

Keep the patient warm and consider warming blood to be transfused.

37
Q

What is the role of folic acid in AIHA management?

A

Folic acid supplements can be given, and folic acid transfusion may be necessary if haemolysis onset is acute.

38
Q

What immunosuppressants are used for AIHA?

A

Rituximab is used for cold/warm haemolysis, and high dose corticosteroids are used for warm haemolysis.

39
Q

What surgical intervention is often needed for warm AIHA?

A

Most warm cases require splenectomy.

40
Q

What is microangiopathic haemolytic anaemia?

A

It is caused by narrowing or obstruction of small blood vessels, leading to intravascular haemolysis.

Examples include Thrombotic thrombocytopenic purpura (TTP), Disseminated intravascular coagulation (DIC), and Haemolytic Uremic Syndrome (HUS).

41
Q

What is Thrombotic thrombocytopenic purpura (TTP)?

A

A rare blood disorder that results in blood clots forming in small blood vessels.

42
Q

What is Disseminated intravascular coagulation (DIC)?

A

A rare blood disorder in which the proteins that control blood clotting become overactive.

43
Q

What can cause repeated mechanical trauma to capillaries?

A

Faulty mechanical heart valves or march haemoglobinuria, which involves repeated force on capillaries in the feet.

44
Q

What are the main investigations for microangiopathic haemolytic anaemia?

A

Urine tests positive for Hb and a negative Coombe’s test, along with a blood film showing schistocytes (fragmented erythrocytes).

45
Q

What infections can cause haemolysis?

A

Infections such as malaria can destroy erythrocytes.

46
Q

What are some chemical and physical agents that can cause haemolysis?

A

Lead, burns, snake and spider bites, and drugs such as dapsone.

This is a rare cause of haemolysis.

Clinical lectures: Yr2 (18 decks)

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