ITP – Idiopathic Thrombocytopenia Purpura

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A 4 year old girl presents with bruising over her legs, trunk and face.  Mum has noticed them appear over the last week.  She has been completely well with no other symptoms.  There is no history of trauma.  After an anxious 1 hour wait the bloods are back – Hb 113, WCC 7.3, Plt 8 x 109/L.

Bottom Line

  • Uncomplicated idiopathic thrombocytopenic purpura (ITP) is new onset bruising and bleeding with a platelet count <100 x 109/L in the absence of other symptoms
  • It generally resolves itself in 80% by six months
  • 5% will have recurrence
  • Only treat if there is active bleeding, not just because of a low platelet count
  • Advise parents to avoid NSAIDS and look out for signs of bleeding
  • Follow up regularly for the first six weeks or until platelet count stabilises

 

What is it and how did she get it?

Idiopathic thrombocytopenic purpura (ITP) is a reduction in platelet count in the absence of any other cause (<100 x 109/L).  Whilst normal platelets last eight to ten days, in ITP there are autoantibodies that destroy them in the first few hours.

It has a peak incidence of two to five years of age (chronic ITP peaks in adolescence).  There is often a recent history (one to six weeks) of a viral illness or immunisation.

What are the commons symptoms and signs?

The most common sign is petechiae (1-5 mm red or purple non-blanching spots) on the skin or mucosa – these indicate capillary haemorrhages.  Some mucocutaneous bleeding is often seen, but it is rare for this to be severe (<5%).

Other symptoms of autoimmune disorders should NOT be present in ITP – e.g. no weight loss, rashes, alopecia, joint swelling.

Examination should be normal with no hepatosplenomegaly or lymphadenopathy.

How is it diagnosed?

It is diagnosed by having a low platelet count with a normal haemoglobin (unlike in leukaemia, TTP, HUS and DIC).

If there is a history of previous bleeding then consider other diagnoses.

Bone marrow aspirate is only recommended if there is persistent bleeding in spite of a platelet count >20 x 109/L.

What treatment should we use?

The answer is simple: treat the patient not the platelet count.  Assess if the patient has haematuria, melaena, menorrhagia, epistaxis, mucosal bleeding or tonsillar purpura/petechiae.

Although there is variation between specialists, they will all be more concerned with the signs of wet purpura or haematuria rather than just the petechiae on the skin.

Steroids

Prednisolone 1-2mg/kg OD for at least three weeks then taper

OR

Methylprednisolone 30mg/kg/day for three days, then 20mg/kg /day for four days

IVIG (intravenous immunoglobulin)

Consider where there is significant bleeding (0.8-1g/kg) – can rapidly raise the platelet count

Effects takes place in one to five days and lasts for two to four weeks

Platelets

Only give platelets if there is ICH or significant bleeding.  Can be effective after IVIG administration and this can prolong platelet survival (otherwise transfused platelets are quickly destroyed)

When to admit?

Admit if there is significant bleeding: epistaxis>1 hour; haematemeses; haemoptysis, intracranial haemorrgae, melaena.  Or if there is an unclear diagnosis or problematic social circumstances.

When will it go away?

Most ITP self resolves.  80% will have resolved by six months (with or without treatment).  5% of ITP patients will have a recurrence.

Although it seems counterintuitive, the lower the platelet count at the beginning, the better.  Uncomplicated ITP normally has a platelet count of <20 x 109/L.

Chronic ITP does not resolve within six months and account for 10% of ITP.

Could it be anything else?

Confirmation is based on excluding other differentials such as acute leukaemia, aplastic anaemia, HUS.  A full blood count and film us usually adequate to make the diagnosis. 

What do you need to inform the parents to look out for?

While the platelets are low, the patient is at risk of bleeding.  ICH is a serious but rare (1%) side effect.  Parents should watch out for any signs of ICH, urinary bleeding, GI bleeding, excessive mucosal bleeding and menorrhagia (in older patients).

They should avoid NSAIDs while the platelet count is low.

Older children should avoid contact sports.  This is completely impractical for young children so is not helpful advice – will only stress out the parents!

When to follow up?

Patients should be reviewed within two weeks of initial presentation and have a repeat FBC.

Aim for weekly GP follow up initially and then PRN until resolution.

Paediatric outpatient review at six weeks three months and six months.

Refer to haematology if unclear diagnosis, unresolved after six months or a haematological malignancy is suggested by the blood count.

 

References

Pediatric EM Morsels – Wet purpura and ITP

UMEM Educational Pearls – ITP

Royal Children’s Hospital, Melbourne – Guidelines for ITP

Princess Margaret Hospital for Children – ITP Guideline

BMJ BestPractice – ITP

Grainger JD, Rees, JL, Reeves M, Bolton-Maggs PHB.  Changing trends in the UK management of childhood ITP. Arch. Dis. Child. 2012;97:8-11.

Parent information sheet

Royal Children’s Hospital Melbourne – ITP for parents

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About 

Tessa Davis is a paediatric emergency registrar from Glasgow and Sydney, but currently living in London. Tessa tries to spend time with her 3 kids in between shifts. @tessardavis | + Tessa Davis | Tessa's DFTB posts

3 Responses to "ITP – Idiopathic Thrombocytopenia Purpura"

  1. Bron Hatton
    Bron Hatton 4 years ago .Reply

    Great to see such common sense explanation about ITP. As an adult with ITP and also a parent of a child with ITP its great to be able to share good info. It sure beats the reactionary comments and over dramatics of some of the US sites. My son currently has a count of 2000 with all but bleeding so we don’t treat. It can be scary as a parent but once you wrap your head around it you can get on with life.Thankyou I have bookmarked this one and printed it out for friend and family reference.

    • Tessa Davis
      Tessa Davis 4 years ago .Reply

      Thanks – interesting to hear from parents as well as medics!

  2. Michelle Bauer
    Michelle Bauer 2 years ago .Reply

    I was diagnosed with ITP at age 2, had a “routine” spleenectony at age 5. My parents were told I was “cured!” Well, it came back with a vengence after my 2nd pregnancy at age 28. I was on steroids constantly, went through multiple chemotherapies, another surgery looking for another slpeen, IVIG gave me aseptic meningitis and landed me in ICU each time. My doctors told me I only had 5 years to live at best. My platelets were 2,000 – 10,000 on my own, I had petechiae and some bruises, and my gums would bleed when brushed but I was not actively bleeding. I was seriously in the hospital more than I was at home. I finally told my doctors I was done taking all their medicine, even steroids! Only my internal medicine doctor has supported me in this, my Hematologist would not see me anymore unless I continued on his course.
    But since I have asked my internist to ONLY TREAT IF ACTIVELY BLEEDING (treat the patient, not.the platelet count), I have stayed out of the hospital (except for blood draws), my platelets have settled between 18,000-24,000 and I am enjoying my children, working part-time as a Registered Nurse and have out-lived those.doctors life expectancy of 5 years by 8 years now.
    This site, don’t forget the bubbles, is right on the button about “treat the patient, not the platelet count”. If your doctor is not willing to listen to what you have.learned/read/heard or try something to help you or your child get better…FIRE HIM! There are more doctors out there! Do your homework and realize that doctors do not always know best. Patient/Doctor relationship should be collaborative, supportive. If not, find one that is. Had I not left my hematologist when I did, I would have been dead 7 years ago.
    Thanks to Tessa who gives her time to get the right information out.

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