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Phase 4 > Haematology > Flashcards

Haematology Flashcards

1
Q

What are one or two symptoms that are characteristic of each leukaemia?

A

AML - gum hypertrophy, rash
CML - massive splenomegaly
ALL - lymphadenopathy, bone/joint pain
CLL - skin lesions (pruritus, herpes zoster)

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2
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked

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3
Q

What is seen on the blood film in G6PD?

A

Bite + blister cells, Heinz bodies

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4
Q

What precipitates an attack in G6PD?

A

Fava broad beans, henna, drugs

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5
Q

Name two enzyme defects that cause haemolytic anaemia?

A

G6PD deficiency

Pyruvate kinase deficiency

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6
Q

Name two membrane defects that cause haemolytic anaemia?

A

Spherocytosis

Elliptocytosis

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7
Q

What happens in spherocytosis?

A

Less flexible spherical RBCs get trapped in the spleen -> extravascular haemolysis

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8
Q

What can cause aplastic anaemia?

A

Idiopathic, congenital, drugs, infections (parvo), radiation

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9
Q

How to Ix aplastic anaemia?

A

BM biopsy

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10
Q

How to Tx aplastic anaemia?

A

Immunosuppression

SC transplant

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11
Q

Presentation of aplastic anaemia?

A

Pancytopaenia

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12
Q

What is the pathophys of B-thalassaemia?

A

There is a loss of beta chains which causes the alpha chains to build up and clump together. This causes the RBC to rupture and release heme which breaks down into unconjugated bilirubin and iron. Causes jaundice and secondary haemochromatosis. Causes hypoxia as fewer RBCs circulating and therefore increased RBC production in BM, liver and spleen (which subsequently enlarge)

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13
Q

What is the treatment of B-thal?

A

No treatment for minor

For major/intermedia -> blood transfusions, iron chelation

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14
Q

What are the Ix for B-thal?

A 
Bloods - low Hb, MCV
raised iron, ferritin, transferrin
Blood smear - microcytic + hypochromic, target cells
Haemoglobin electrophoresis
Head X-ray - hair-on-end appearance
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15
Q

Presentation of alpha-thal?

A

Anaemia
Hepatosplenomegaly
Skeletal abnormalities

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16
Q

What happens in sideroblastic anaemia?

A

RBCs fail to completely form haem, so iron gets deposited into the mitochondria

17
Q

Causes of sideroblastic anaemia?

A

Congenital (microcytic)

Acquired (alc, lead, TB meds) - macrocytic

18
Q

Ix for sideroblastic anaemia?

A

Blood film, BM biopsy

19
Q

Tx for sideroblastic anaemia?

A

Supportive - blood transfusions, abx, iron chelation
Treat underlying cause
Pyridoxine

20
Q

Sickle-cell anaemia pathophys?

A

Point mutation in codon 6 in B-globin gene

Coded AA changes from glutamine -> valine

21
Q

Presentation of sickle-cell disease?

A

Anaemia symptoms
Infections d/t hyposplenism
Priapism
Splenomegaly
Hand-foot syndrome - dactylitis, swelling, pain
Acute chest syndrome/limb/spine bone occlusions

22
Q

How to treat acute SCA?

A

Analgesia, fluids, O2, abx, blood transfusion, exchange transfusion

23
Q

How to treat SCA?

A

Hydroxyurea, vaccinations

24
Q

SCA investigations?

A

Bloods, blood film, sickle solubility test, Hb electrophoresis

25
Q

What is the triad presentation of pernicious anaemia?

A

Anaemia
GI disturbances - diarrhoea, constipation, loss of bladder/bowel function
Neuro disturbances

26
Q

How to Ix pernicious anaemia?

A 
Bloods
Low serum B12
Parietal cell/IF Abs
Blood film
BM biopsy
27
Q

Tx of pernicious anaemia?

A

3 monthly B12 injections +/- folic acid supplements

28
Q

Source of folate?

A

Green vegetables, liver, nuts

29
Q

Treatment of folate deficiency?

A

Folic acid supplements

Give B12 also if low to avoid subacute combined degeneration of the spinal cord

30
Q

What two things can polycythaemia vera turn into?

A

Myelofibrosis, AML

31
Q

What are the symptoms of polycythaemia vera?

A
  • Hyper-viscosity
  • Red/red-blue tinge of nose, ear, lips, mouth
  • Pruritis, typically after a hot bath (due to histamine release from excess basophils and mast cells)
  • Splenomegaly
  • Gout, kidney stones

Phase 4 (13 decks)

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