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Pathology eLearning - Y5 > Haemolytic Anaemia > Flashcards

Haemolytic Anaemia Flashcards

1
Q

What is the lifespan of a normal red blood cell?

A

120 days

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2
Q

What are different categories of haemolysis?

A
  • Intravascular: Within circulation
  • Extravascular: Removal/destruction by reticuloendothelial (RE) system.

Inherited or acquired

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3
Q

What are causes of extravascular haemolytic anaemia?

A

Autoimmune

Alloimmune

Hereditary spherocytosis

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4
Q

What are causes of intravascular haemolytic anaemia?

A
  • Malaria
  • G6PD deficiency
  • Mismatched blood transfusion (ABO)
  • Cold antibody haemolytic syndromes
  • Drugs
  • Microangiopathic haemolytic anaemia
    • Haemolytic uraemic syndrome
    • Thrombotic thrombocytopenic purpura
  • Paroxysmal nocturnal haemoglobinuria
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5
Q

What are causes of hereditary haemolytic anaemia?

A

Disorders of:

Membrane

  • Cytoskeletal proteins
  • Cation permeability

Red cell metabolism

Haemoglobin:

  • Thalassaemia
  • Sickle cell syndromes
  • Unstable Hb variants
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6
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

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7
Q

What are consequences of haemolytic anaemia?

A

Anaemia(+/-)

Erythroid hyperplasia with increased rate of red cell production and circulating reticulocytes

Increased folate demand

Susceptibility to effect of parvovirus B19

Propensity to gallstones(cholelithiasis)

Increased risk of:

  • Iron overload
  • Osteoporosis
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8
Q

What is this?

A

Parvovirus B19 infection

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9
Q

What is this?

A

Hepatic siderosis (Perl’s stain)

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10
Q

What does coinheritence of Gilbert syndrome with chronic haemolytic anaemia lead to increased risk for?

A

Cholelithiasis

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11
Q

What are clinical features of haemolytic anaemia?

A

Pallor

Jaundice

Splenomegaly

Pigmenturia

Family history

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12
Q

What are laboratory findings of haemolytic anaemia?

A

Anaemia

Increased reticulocytes

Polychromasia

Hyperbilirubinaemia

Increased LDH

Reduced/absent haptoglobins

Haemoglobinuria

Haemosiderinuria

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13
Q

What are defects in red cell membrane disorders in hereditary spherocytosis?

A

Vertical interaction:

  • Band 3
  • Protein 4.2
  • Ankyrin
  • Beta-Spectrin
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14
Q

What are defects in red cell membrane in hereditary elliptocytosis?

A

Horizontal interaction:

  • Alpha-Spectrin
  • Beta-Spectrin
  • Protein 4.1
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15
Q

What is hereditary spherocytosis?

A

Genetic defect of red cell cytoskeleton.

  • Family history in 75% - typically autosomal dominant
  • 25% recessive or de novo mutation

In vitro red cells show increased sensitivity to lysis in hypotonic saline (osmotic fragilty test).

Reduced binding of dye eosin-5-maleimide.

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16
Q

What is this?

A

Hereditary spherocytosis (HS)

17
Q

What is this?

A

Hereditary elliptocytosis

18
Q

What is this?

A

Hereditary pyropoikilocytosis

19
Q

What is the epidemiology and pattern of inheritence of Glucose-6-phosphate dehydrogenase deficiency?

A

Affects up to 400 million worldwide.

Prevalent in areas of malarial endemicity - selection.

X-linked - clinical effects seen predominantly in hemizygous males and homozygous females.

20
Q

What is the pathophysiology of G6PD deficiency?

A

Enzyme catalyses first step in pentose phosphate (hexose monophosphate) pathway - generates NADPH required to maintain intracellular glutathione (GSH).

21
Q

What are the clinical effects of G6PD deficiency?

A

Neonatal jaundice

Acute haemolysis (triggered by oxidants/infection)

Chronic haemolytic anaemia (rare)

22
Q

What triggers symptoms of G6PD deficiency?

A

Steady state is asymptomatic, however, drugs, infections or fava beans can lead to acute haemolysis.

23
Q

What is this?

A

G6PD deficiency

24
Q

What is this?

A

Heinz bodies (Methylviolet)

25
Q

Which agents can provoke acute haemolysis in G6PD deficiency?

A

Anti-malarials: Primaquine

Antibiotics:

  • Sulphonamides
  • Ciprofloxacin
  • Nitrofurantoin

Other drugs:

  • Dapsone
  • Vitamin k

Fava beans

Mothballs

26
Q

What is involved in Embden-Meyerhof part of glycolysis?

A

NADPH

ATP

27
Q

What is involved in Hexose monophosphate shunt (pentose phosphate) part of glycolysis?

A

NADPH

28
Q

What is involved in Rapoport-Luebering shuttle part of glycolysis?

A

2,3-DPG

29
Q

What is involved in nucleotide metabolism part of glycolysis?

A

Adenine salvage

Adenosine phosphate equilibrium

Removal of pyrimidine nucleotides

30
Q

What is involved in glutathione biosynthesis part of glycolysis?

A

GSH

31
Q

What is involved in cytochrome b5 reductase part of glycolysis?

A

Methemoglobin reduction

32
Q

What is this?

A

Pyruvate kinase deficiency (post-splenectomy)

33
Q

What is this?

A

Pyrimidine 5’-nucleotidase deficiency

34
Q

What are the first line investigations for G6PD deficiency?

A
  • Direct antiglobulin test
  • Urinary haemosiderin/haemoglobin
  • Osmotic fragility
  • G6PD +/- PK activity
  • Haemoglobin separation A and F%
  • Heinz body stain
  • Ham’s test/Flow cytometry of GPI-linked proteins
  • Thick and thin blood film
35
Q

What are the principles of management of G6PD deficiency?

A
  • Folic acid supplementation
  • Avoidance of precipitating factors e.g. oxidants in G6PD deficiency
  • Red cell transfusion/exchange
  • Immunisation against blood borne viruses e.g. hepatitis A and B
  • Monitor for chronic complications
  • Cholecystectomy for symptomatic gallstones
  • Splenectomy if indicated
36
Q

What are indications for splenectomy?

A
  • PK deficiency and some other enzymopathies
  • Hereditary spherocytosis
  • Severe elliptocytosis/pyropoikilocytosis
  • Thalassaemia syndromes
  • Immune haemolytic anaemia
37
Q

What are risks of splenectomy?

A

Risk of overwhelming sepsis:

  • Capsulated bacteria e.g. Pneumococcus
  • Penicillin prophylaxis and immunisation
38
Q

What is the criteria for splenectomy?

A

Transfusion dependence

Growth delay

Physical limitation Hb

Hypersplenism

Age not < 3 years, before 10 years to maximise prepubertal growth.

Pathology eLearning - Y5 (17 decks)

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