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Fourth Year > Haematology > Flashcards

Haematology Flashcards

1
Q

Mode of inheritance of Haemophilia A?

A

X-linked recessive.

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2
Q

Haemophilia A is a deficiency in what?

A

Coagulation factor VIII.

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3
Q

Most common form of haemophilia?

A

Haemophilia A.

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4
Q

Haemophilia B is a deficiency of?

A

Factor IX.

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5
Q

How does haemophilia present?

A
  • Prolonged bleeding after minor trauma.
  • Haematoma formation.
  • Spontaneous bleeding into joints.
  • Soft tissue haemorrhage.

SEVERE:

  • Haematuria.
  • GI haemorrhage.
  • IM and/ or intracranial haemorrhage.
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6
Q

Describe the bleeding time seen in haemophilia A.

A

Normal.

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7
Q

Describe prothrombin time seen in haemophilia A.

A

Normal PT.

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8
Q

Describe activated partial thromboplastin time seen in haemophilia A.

A

Prolonged APTT.

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9
Q

How is haemophilia A managed?

A

Regular Factor VIII infusions.

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10
Q

Von Willebrand disease is a deficiency in what?

A

Von Willebrand factor.

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11
Q

Functions of von willebrand factor (2)?

A
  • Enhances platelet adhesion to areas of endothelial damage and vascular injury.
  • Stabilises factor VIII by binding to it.
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12
Q

When does Von Willebrand disease present?

A

At any point in life.

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13
Q

Symptoms of Von Willebrand Disease?

A
  • Easy bruising.
  • Prolonged bleeding following minor trauma.
  • Heavy bleeding following an operation.
  • Conjunctival bleeding.
  • Umbilical stump bleeding.
  • Menorrhagia.
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14
Q

Blood results of Von Willebrand disease?

A
  • Prolonged bleeding time.
  • Mildly prolonged APTT.
  • Normal PT.
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15
Q

How is Von Willebrand disease managed?

A
  • Desmopressin.

- Recombinant vWF or a combo of vWF and factor VIII.

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16
Q

Blood test results indicative of Vitamin K deficiency?

A
  • Prolonged PT.
  • Normal APTT, fibrinogen concentration and platelet count.
  • Low Vitamin K-dependent coagulation factors.
17
Q

Which coagulation factors are vitamin K dependent?

A

II, VII, IX, X.

Proteins C and S.

18
Q

Paediatric liver cirrhosis in infants is most commonly due to?

A

Biliary atresia.

Inherited metabolic diseases.

19
Q

Paediatric liver cirrhosis in older children is most commonly manifestations of which diseases?

A
  • Autoimmune hepatitis.
  • Primary sclerosing cholangitis.
  • Viral hepatitis.
  • Wilson’s disease.
20
Q

How might you support a diagnosis of childhood cirrhosis?

A
  • Deranged LFTs

+ prolonged PT not corrected despite adequate vitamin K administration (indicated poor liver synthetic function).

21
Q

What is idiopathic (immune) thrombocytopenic purpura?

A

Low number of platelets due to autoimmune destruction.

22
Q

How does ITP present?

A
  • Bleeding, bruises and petechiae.
23
Q

Blood results suggestive of ITP?

A
  • Thrombocytopenia (low platelet count) - usually isolated.
  • Normal Hb.
  • Normal leukocytes.
24
Q

Vitamin B12 deficiency causes what?

A

Megaloblastic anaemia.

25
Q

Cause of pernicious anaemia?

A

Atrophy of gastric mucosa > subsequent reduction in production of intrinsic factor (required for B12 absorption in ileum).

26
Q

Why does reduced intrinsic factor result in B12 deficiency?

A

Intrinsic factor is required for B12 absorption in the ileum.

27
Q

Blood / bone marrow results of someone with pernicious anaemia?

A
  • Raised MCV.

- Megaloblasts in bone marrow.

28
Q

Causes of fragment cells?

A
  • Prosthetic heart valves.
  • Renal dialysis.
  • Microangiopathic haemolytic anaemias.
29
Q

Sickle cell crises may be spontaneous or initiated by?

A
  • Infection.
  • Cold.
  • Hypoxia.
30
Q

Symptoms of sickle cell?

A
  • Chest pain.
  • Bone pain.
  • Priapism.

More severe:

  • Neurological sequelae.
  • Hyposplenism (recurrent splenic infarcts).
31
Q

Rheumatoid arthritis + hypersplenism + neutropenia.

A

Felty’s syndrome.

> Neutropenia with myeloid hyperplasia.

32
Q

Blood results of someone with lead poisoning?

A
  • Sideroblasts.

- Basophilic stippling.

33
Q

Effects of lead poisoning?

A
  • Inhibition of enzymes involved in haem synthesis > anaemia.
  • Inhibition of enzymes dispersing excess RNA > stippling (discrete blue particles).
34
Q

Describe the abnormal blood results of a patient with hookworm infection.

A
  • Hypochromia.
  • Anisocytosis.
  • Poikilocytosis.
    Due to iron deficiency anaemia - hookworm infestation of duodenum is the most common infective cause worldwide.
35
Q

Causes of iron deficiency anaemia?

A
  • Blood loss.
  • Poor dietary intake.
  • Decreased absorption.

Fourth Year (28 decks)

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