diagnosis and management of typical, newly diagnosed primary ITP of childhood

Question 1

Red flags for ITP?

Answer 1

• history
  
  • constitutional symptoms (fevers, wt loss, night sweats)
  • bone pain
  • recurrent thrombocytopenia
  • poor treatment response
• Physical exam
  
  • lymphadenopathy
  • hepatomegaly
  • splenomegaly
  • child “unwell”
  • signs of chronic disease
• Investigations
  
  • Low Hb (unless mildly low and explained by bleeding)
  • high MCV
  • abnormal WBC and/or neutrophil count
  • abnormal cellular morphology on smear

Question 2

What are secondary causes of ITP?

Answer 2

drug-induced, SLE, infections, immunodeficiencies, malignancy

Question 3

When does ITP typically resolve by?

Answer 3

6 months (75-80% of cases resolve in that time)

Question 4

What is the typical age range for ITP?

Answer 4

2-5 yrs

Question 5

What is the most serious complication of ITP?

Answer 5

ICH (0.17-0.6%)

Question 6

What should be done to treat a child with:

• no active bleeding
• moderate bleeding
• severe bleeding

Answer 6

• Children without active bleeding:
  
  • first line: observation
  • second line: oral steroids or IVIG
• Children with moderate bleeding
  
  • First line: single dose IVIG (0.8-1 g/kg) or short course steroids
  • anti-D (IV immune globulin) only for Rh positive children. Not first line because of serious AE (possible DIC)
• Children with severe bleeding
  
  • prolonged epistaxis, GI bleeding or ICH (an estimated 3% of total cases)
  • hospital admission for IV steroids and IVIG
  • Tranexamic acid (25 mg/kg/dose administed 3-4/day to max 1500 mg/dose)

ULTIMATELY, any single option doesn’t clearly outweigh potential risks

Question 7

ITP -

what is considered no or mild bleeding?

Answer 7

no bleeding or (at most) bruising, petechiae, occasional mild epistaxis
No or little interference with daily living
includes non-oozing petechiae on oral mucosa or resolved mild epistaxis

• 77%

Question 8

ITP -

What is considered moderate bleeding?

Answer 8

more severe skin manifestations with some mucosal lesions and more troublesome epistaxis or menorrhagia

• 20%

Question 9

ITP -

What is considered severe bleeding?

Answer 9

bleeding episodes (epistaxis, melena, menorrhagia and/or intracranial hemorrhage) requiring hospital admission

• 3%
• plt transfusion contraindicated except for acute, life-threatening bleeds or children needing immediate surgery

Question 10

how quickly do steroids work in ITP?

Answer 10

Effective 72-88% cases

increase in plts, usually within 48 hr

Question 11

how quickly does IVIG work in ITP?

Answer 11

Effective in >80% cases
Increase plt within 24 hr, peak at 2-7 days
Use if rapid increase required

Question 12

what percent of kids relapse with ITP?

Answer 12

• 1/3 of children who respond to treatment will relapse with plt counts <20 x 10^9 within 2-6 weeks

Question 13

What are the supportive care & monitoring recommendations for kids with ITP?

Answer 13

• regular appointments until plt counts recovered
• when plt counts remain low or evidence or bleeding, avoid sports and activitie that may cause injuries (esp to head)
• avoid NSAIDs and herbal products