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Medicine: Haematology > Haemolytic Anaemia > Flashcards

Haemolytic Anaemia Flashcards

1
Q

Where does haemolysis occur?

A
  1. Circulation (intravascular)

2. Reticuloendothelial system - macrophages of liver, spleen and bone marrow (extravascular)

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2
Q

What blood/urinary tests would indicate an increased RBC breakdown?

A
  1. Anaemia with normal or raised MCV
  2. Raised bilirubin - unconjugated
  3. Increased urinary urobilinogen
  4. Increased LDH (non-specific)
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3
Q

What blood tests could indicate an increased RBC production?

A

Increased reticulocytes (large immature RBCs) causing raised MCV.

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4
Q

What are the features of intravascular haemolysis?

A
  1. Decreased haptoglobin (mops up free Hb)
  2. Haemoglobinuria (red-brown urine, absence of RBCs)
  3. Haemosidinuria (chronic intravascular haemolysis)
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5
Q

What would the results of these tests in intravascular vs extravascular haemolysis?

  1. Unconjugated bilirubin
  2. LDH
  3. Reticulocytes
  4. Haptoglobin
  5. Haemoglobinuria (+/-)
  6. Haemoglobinaemia (+/-)
  7. Hemosiderinuria (+/-)
A 
Intravascular:
1. High
2. High
3. High
4. Low
5. +
6. +
7. +
Extravascular:
1. High
2. High
3. High
4. Normal
5. -
6. -
7. -
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6
Q

What are the causes of haemolytic anaemia?

A
  1. Acquired - autoimmune, alloimmune, microangiopathic, paroxysmal haemoglobinuria.
  2. Inherited - G6PD deficiency, pyruvate kinase deficiency, membrane defects, haemoglobinopathies.
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7
Q

What are the two types of autoimmune haemolytic anaemia?

A
  1. Warm AIHA - IgG mediated, DAT +ve, idiopathic
  2. Cold AIHA - IgM mediated, causes chronic anaemia
  3. Paroxysmal cold haemoglobinuria - seen with viral infections
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8
Q

What are the treatments for the two types of autoimmune haemolytic anaemia?

A
  1. Warm AIHA - steroids and immunosuppression

2. Cold AIHA - keep warm

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9
Q

What are the causes of alloimmune haemolytic anaemia?

A
  1. Blood transfusion reaction

2. Haemolytic disease of the newborn

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10
Q

What is microangiopathic haemolytic anaemia and what causes it?

A
  1. Mechanical damage to RBCs in circulation

2. TTP, DIC, prosthetic heart valves

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11
Q

What is this describing and what is the treatment?
Pentad: fever, renal failure, confusion, thrombocytopenia. Endothelial damage releases vWF causing platelet aggregation. Microangiopathic haemolytic anaemia.

A
  1. Thrombotic thrombocytopenic purpura (TTP)

2. Plasma exchange, steroids, blood, folic acid

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12
Q

What is this describing?
Systemic activation of coagulation pathways. Fibrin clots generated = organ failure. Consumption of platelets = clinical bleeding. Microangiopathic haemolytic anaemia. Increased PT, aPTT, D-dimer. Reduced fibrinogen, platelets. Caused by sepsis, trauma, cancer, obstetrics.

A

Disseminated intravascular coagulopathy

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13
Q

What is this describing?
X-linked RBC enzyme defect. Males, Mediterranean, Africa, Far-East. Oxidative crisis due to low glutathione. Precipitated by aspirin and antimalarials. Rapid anaemia and jaundice in attacks.

A

Glucose-6-phosphate dehydrogenase deficiency

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14
Q

What is the inheritance pattern of pyruvate kinase deficiency?

A

Autosomal recessive

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15
Q

What are the main membrane defects causing inherited haemolytic anaemia, what is their pattern of inheritance, and do they cause intra- or extravascular haemolysis?

A
  1. Hereditary spherocytosis (AD)
  2. Hereditary elliptocytosis (AD)
    Extravascular haemolysis
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16
Q

What are the two inherited haemoglobinopathies that cause haemolytic anaemia?

A
  1. Sickle cell

2. Thalassaemia

17
Q

What is direct Coombs test?

A
  1. Blood sample from patient with immune haemolytic anaemia taken.
  2. RBCs washed and incubated with antihuman antibodies.
  3. RBCs agglutinate via antihuman antibodies in a positive Coombs test.
18
Q

What is indirect Coombs test?

A
  1. Recipients serum obtained containing antibodies.
  2. Donor’s blood sample added to tube with serum.
  3. Recipients Igs that target donor’s RBCs form complexes.
  4. Anti-human Igs added.
  5. Agglutination of RBCs occurs due to anti-human antibodies (positive test).

Medicine: Haematology (18 decks)

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