purpura

Question 1

A 4 year old boy presents with a 24 hour history of rash and easy bruising. He is otherwise well. He had a cough, coryza and fever one month previously. There is no past medical history or family history of a bleeding disorder.

What do you observe on his face?

Answer 1

purpura

Question 2

This is a picture of an older child with the same illness, what can you see?

Answer 2

purpura

Question 3

Blood tests show:

• Platelet count 15 x10⁹
• Hb 115 g/L
• WCC 10.6 x10⁹
• Blood film: platelets decreased in number but normal size, white and red blood cells normal

What is the most likely diagnosis?

Answer 3

immune thrombocytopenic purpura

Question 4

Should all children have a bone marrow examination?

Answer 4

no, performed if atypical to look for evidence of:

• malignancy (ie if any lymphadenopathy, hepatosplenomegaly, bone or joint pain, fever, weight loss, neutropenia, leucocytosis, immature lymphocytes (blasts) on blood film)
• bone marrow failure; failure to resolve spontaneously (not true indication but some centres perform before steroid treatment); insufficient response to treatment with steroids, IVIG).

Question 5

What is the risk of intracranial haemorrhage?

Answer 5

<1%

Question 6

What are the treatment options?

Answer 6

• children with no or mild bleeding (cutaneous only) can be managed conservatively with a ‘watch and wait’ approach.
• should avoid:
  
  • high risk activities such e.g. contact sports
  • antiplatelet medications e.g. ibuprofen
• if mucosal bleeding / suspected internal heamorrhage / life-threatening bleeding:
  
  • IVIG
  • steroids
  • platelet transfusions
  • splenectomy

Question 7

What is the likely prognosis?

Answer 7

majority resolve within 3-6 months

80-90% by 6 months

Question 8

A 7 year old boy presents with a symmetrical rash over the extensor surfaces of his legs, with pain and swelling in both ankles. He had a cough, coryza and fever two weeks previously.

On examination the rash is non-blanching and palpable.

What is the most likely diagnosis?

Answer 8

Henoch-Schonlein purpura

Question 9

What three other organ systems may be involved?

Answer 9

MSK

GI

renal

Question 10

Which investigations should be performed?

Answer 10

1. BP
2. urine dipstick
3. protein:creatinine ratio (if protein ++ on dipstick)
4. renal function
5. FBC, blood film, coag screen - to exclude other ddx

Question 11

What are the recommended treatment options?

Answer 11

• supportive: pain mx as required with paracetamol / NSAIDs
• severe: steroids
• regular monitoring of BP and urinalysis

Question 12

What are possible side effects of steroid treatment?

Answer 12

• hyperglycaemia
• HTN
• wt gain
• acne
• immunosuppression

Question 13

What is the usual prognosis?

Answer 13

• initial episode: usually resolves within 1 month
• 1/3: recurrence
• 20-50% have renal involvement at initial presentation
  
  • 90% who develop renal disease do so within 2 months of onset