Diagnosis and management of ITP Flashcards

1
Q

What can trigger ITP?

A

Viral infection or immune phenomenon

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2
Q

What does ITP stand for?

A

Primary Immune Thrombocytopenia

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3
Q

Who does ITP affect?

A

5 in 100 000 children

Usually between 2-5 years of age

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4
Q

What is the natural history of ITP?

A

Usually self resolving within 6 months for 75-80% of cases

- Remaining children usually resolve within the year

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5
Q

How do most children present with ITP?

A

Bruising and petechiae

- 3% will have a more severe presentation* including bleeding nose, mucosa, or GI tract

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6
Q

What is the most serious complication of ITP? What is the incidence of this?

A
  • Intracranial hemorrhage

- 0.17-0.6% of cases

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7
Q

What are red flags on history for a child with suspected ITP?

A

Constitutional symptoms (fevers, weight loss, night sweats) Bone pain
Recurrent thrombocytopenia
Poor treatment response

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8
Q

What are red flags on physical exam for a child with suspected ITP?

A
Lymphadenopathy 
Hepatomegaly 
Splenomegaly
Child is “unwell”
Signs of chronic disease
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9
Q

What are red flags with investigations for a child with suspected ITP?

A

Low Hb (unless mildly low and explained by bleeding history)
High mean corpuscular volume (MCV)
Abnormal white blood cell (WBC) and/or neutrophil count Abnormal cellular morphology on smear

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10
Q

What should be done if red flags are present?

A

Urgent referral to Hematology

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11
Q

What is the definition of ITP?

A

Platelet count <100 x 10^9/L

Most patients will have <20 x 10^9/L

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12
Q

Is a bone marrow necessary for children with ITP?

A

No* if there are no red flag features present

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13
Q

What are secondary causes of ITP to consider?

A
Drug induced
SLE
Infections
Immunodeficiencies 
Malignancy
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14
Q

How to do pharmacologic treatments for ITP work?

What are some disadvantages to consider?

A
  • Increase platelet count and reduce perceived bleeding risk
  • Downsides include possible hospitalization and side effects from exposure to medication
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15
Q

What are treatment options for ITP?

A
  • Observation (+/- treatment)
  • Corticosteriods
  • IVIG
  • Anti-D immunoglobulin (Anti-D) for patients that are Rh-positive children
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16
Q

Who can the American Hematology Society guidelines be applied to?

A

Any child between 90 days to 17 years of age
Newly diagnosed primary ITP (up to three months post diagnosis)
*not meant to be applied to chronic, secondary or persistent ITP

17
Q

Is treatment for ITP curative?

A

NO!

18
Q

Based on the new (2011) guidelines, what is the goal of treatment with ITP?

A

Achieve a platelet count associated with adequate hemostasis ie: no active bleeding, no epistaxis, no menorrhagia or blood within the stool
no absolute platelet number as target

19
Q

What is considered mild bleeding with ITP? What percentage of children have mild symptoms?

A

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

  • 77% of cases
20
Q

What is the goal of treatment with ITP?

A

To raise platelet level high enough to obtain hemostasis

21
Q

What is considered moderate bleeding with ITP? What percentage of children have moderate symptoms?

A

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

Seen in 20% of cases

22
Q

What is considered severe bleeding with ITP? What percentage of children have severe symptoms?

A

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

Seen in 3% of cases

23
Q

What is the treatment for mild ITP?

A

1) Observation

2) Second line treatment can include IVIG or corticosteroid course

24
Q

What is the treatment for moderate ITP?

A

1) IVIG (0.8 to 1 g/kg)
2) Short course of steroids
3) Anti-D immunoglobulin for those who are Rh-positive*
* not considered first line due to rare but serious side effects that can occur

25
Q

What is the treatment for severe ITP?

A
  • Immediate treatment in hospital with intravenous steroids and IVIG is indicated
  • Tranexamic acid may help as an adjunct therapy at a dose of 25 mg/kg/dose administered 3 to 4 times per day (to a maximum of 1500 mg per dose or 4500 to 6000 mg per day)
  • Life-threatening: IV methylprednisolone + IVIG + platelet transfusion +/- tranexamic acid

**Platelet transfusion is generally contraindicated except for acute, life-threatening bleeds or in children requiring immediate surgery

26
Q

Should platelets be used for treatment of ITP?

A

Generally, NO!

**Platelet transfusion is generally contraindicated except for acute, life-threatening bleeds or in children requiring immediate surgery.

27
Q

Why should a shared decision model be used with the treatment of ITP?

A

Relative rarity of severe bleeding with ITP makes it unlikely that future trials will fully establish the risks and benefits of active treatment or alleviate the decisional conflict that exists among providers and families considering treatment options

28
Q

What factors should be considered when deciding treatment course for ITP?

A

Risk adversity
The desire for inpatient versus outpatient management, Risks and benefits of each option (e.g., degree of comfort with blood products or corticosteroid side effects, and observation versus active therapy

29
Q

What percentage of children will relapse after treatment?

A

1/3 of children will initially respond (regardless of treatment type) but will relapse with platelets count falling below 20 x10^9L/min within 2-6 weeks

30
Q

What should patients avoid if their platelet counts are low or they have evidence of bleeding?

A

Sports or activity with potential for injury* especially head injury
NSAIDs
Herbal supplements

31
Q

What steroid dose can be used for moderate ITP?

A

Prednisone 4 mg/kg/day orally divided TID or QID for 4 days (to a maximum 150 mg/day without taper
OR
2 mg/kg/day orally for 1 to 2 weeks, with tapering dose

No evidence for shorter vs longer course

32
Q

What are some disadvantages to observation?

What are some advantages?

A

Longer period of activity restriction
Anxiety while awaiting platelet recover

No medication cost and outpatient management

33
Q

What are some disadvantages to steroid treatment?

What are some advantages?

A

Mood changes
Increased appetite, weight
Gastritis (consider use of stomach protectant) Hypertension (with a longer course)
Poor taste may affect tolerance

Outpatient management with no IV
Inexpensive

34
Q

What is the dose of IVIG?

A

0.8 to 1 g/kg per day

Consider measuring baseline IG levels for children <1 year of age or when an immune deficiency is suspected

35
Q

What are some disadvantages to IVIG?

A
Headache (aseptic meningitis)
Nausea, vomiting Fever
Rash
Hemolysis (rare)
Requires IV placement and (at minimum) a one-day stay in hospital

Also expensive!

36
Q

What is the effectiveness of observation as a treatment?

A

75% to 80% will improve within 6 months

Will take longer for platelet count to rise

37
Q

What is the effectiveness of steroids as a treatment?

A

Effective in up to 72% to 88% of cases

Increase in platelets, usually within 48 h

38
Q

What is the effectiveness of IVIG as a treatment?

A

Effective in >80% of cases
Increase in platelets usually within 24 h; peak response at 2 to 7 days
Should be used if more rapid increase is required