Haem - ITP, Aplastic Anaemia

Question 1

Immune (or idiopathic) thrombocytopenic purpura (ITP) - what is it?

Answer 1

Autoimmune disease of unknown cause where the number of circulating platelets is reduced

Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex

Question 2

ITP - how doe sit present in children and adults?

Answer 2

In adults more of a chronic condition

In children - usually acute thrombocytopenia following infection or vaccination

Question 3

ITP in adults - what is the clinical presentation?

Answer 3

Symptomatic patients:

1. petichae, purpuric rash (non-blanching rash)
2. bleeding (e.g. epistaxis)
3. catastrophic bleeding (e.g. intracranial) - not common

Can be found incidentally on routine bloods

Question 4

ITP in adults - management?

Answer 4

1st LINE - Oral Prednisolone

Pooled normal human immunoglobulin (IVIG) - raises platelet count faster than steroids, so use in active bleeding or urgent invasive procedure

Question 5

Aplastic Anaemia - what is it?

Answer 5

Characterised by pancytopenia and a hypoplastic bone marrow

Question 6

Aplastic Anaemia - peak age of incidence?

Answer 6

30 years old

Question 7

Aplastic Anaemia - clinical features?

Answer 7

NORMOCHROMIC, NORMOCYTIC ANEMIA

leukopenia, with lymphocytes relatively spared

thrombocytopenia

may be the presenting feature acute lymphoblastic or myeloid leukaemia

a minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia1.

Question 8

Aplastic Anaemia - causes?

Answer 8

idiopathic

drugs: cytotoxics, chloramphenicol, sulphonamides, PHENYTOIN, gold
toxins: benzene
infections: parvovirus, hepatitis

radiation

congenital: Fanconi anaemia, dyskeratosis congenita

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