Thank you for all your help this week!
This week we looked at a case of childhood ITP.
Our patient presented with easy bruising over a 3 week period, and had had a minor urti the previous month.
The patient had no sweats, no bone pains and had otherwise been feeling read only well, except she had been slightly tired over the last couple of weeks.
On examination she had some bruising and a slight petichial rash on her legs. There was I evidence of any oral bleeding. No splenomegally, no lymphadenopathy.
The patient was only taking multivitamins and no other medications.
The was no family history of bleeding problems and the girl had not previously had any problems with bleeding.
Initial bloods revealed
Pregnancy test negative
HIV/hep b/hep b core/hep c negative
A blood film showed occasional large platelets. No fragment, normal neutrophils and lymphocytes and no blasts.
The patient was given some oral iron replacement. And the platelet count was observed.
No treatment was initiated but the patient and parents were advised that if there were and bleeding problems they should attend. The patient had been advised that she should avoid any contact sports.
The following week the platelet count had improved to 17. And following that 35.
Unfortunately while the patient was taking part in running at school she got hit in the nose by another pupil. She had epistaxis for 15 mins and the nose bleed settled her parents were contacted and she was taken to hospital for assessment. On the way she had a further small nose bleed for 2 mins. A repeat plt count at this time showed a plt count of 40. The patient was given tranexamic acid and observed. She had no further bleeding so was discharged but advised to re-attend in any further bleeding and given tranexamic acid orally for 7 days.
A platelet count in the subsequent 2 weeks was 90 and 150.
Immune thrombocytopenia is an acquired thrombocytopenia mediated by autoantibodies causing decreased circulating platelet survival.
Most patients will have a self limiting course which will usually resolve in under 6 months, but usually by 2-3 months. This is often preceded by a viral illness. Around 10% of patients may have a more chronic course when the thrombocytopenia does not resolve by 6 months.
Children will usually present with bruising and petichial rash, but can also have oral bleeding/epistaxis/haematuria/rectal bleeding.
The risk of morbidity and mortality from childhood ITP is very low. The risk of ICH in children with ITP has been found to be <0.2% in several studies. (1,2,3)
When thrombocytopenia is found in a child a thorough history and examination should be done. It is important to consider other causes for thrombocytopenia including malignancies, bone marrow failure syndromes, and congenital thrombocytopenias.
If there are doubts as to the diagnosis of ITP based on history, examination or laboratory findings then a bone marrow should be considered. It is important to ask about symptoms such as bone pains, sweats, weight loss and to elicit and evidence of lymphadenopathy or organomegally on examination which may point you in the direction of alternative diagnosis such as malignancies.
A family history should enquire as to any other medical problems, or thrombocytopenias/ bleeding problems which may indicate an inherited platelet disorder.
Following the assessment management will be influenced be a number of factors.
If the patient has no bleeding, regardless of platelet count, then they may be conservatively managed this can be as an outpatient providing adequate information is given to the parents/carers/child regarding the condition and representing if there are any concerns or signs of bleeding. Robust follow up and instructions on where to present if problems occur should also be in place.
Advice should be given that they should avoid doing any contact sports or things which may result in injury such as trampolining!
Advice on avoiding aspirin and NSAIDs should be given.
If the child is bleeding then treatment should be considered.
Very minor bleeding could be treated with tranexamic acid, but children should be assessed to ensure they do not need systemic treatment.
Treatment options can include IV immunoglobulin. This usually has a rapid onset of response, but response is usually transient and may only last for a few weeks. Which given the majority of patients may have a transient course lasting only a few weeks to months may be enough.
Corticosteroids are also a treatment option however long term corticosteroid use should be avoided in children with acute ITP due to side effects.
Anti D has also been used in non splenectomised children with ITP who are Rh positive who have a negative DAT. There is a risk of intravascular haemolysis with this however, so benefits should be weighed against the risks of using this by a clinician experienced in managing ITP. If used patients will need to be monitored for haemolysis.
For children who are none responders to first line treatment or who have chronic ITP where quality of life is affected rituximab can be used. It is important to ensure the patient has been tested for hepatitis b (including hep b core) as there have been reports of reactivation of hepatitis B. If a response is gained this may typically last for 12-18 months.
Other immunosuppressants such as MMF have also been used in children. TPO agonists such as elthrombopag are also licensed for children with ITP.
Splenectomy can also be considered for children who have severe disease who are none responders to other treatments. This is usually delayed as there is a high rate of spontaneous remission in children with ITP.
Relapse of ITP can happen and this may be precipitated by a viral illness. Patients and their parents/carers should be made aware of this and advised to represent if further symptoms.
Thankyou for all your input this week! Is there anything you want to ask? Anything you would do differently?
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1. Rosthøj S, Hedlund-Treutiger I, Rajantie J, et al. Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: a prospective nordic study of an unselected cohort. J Pediatr2003;143(3):302-307.
2. Kühne T, Buchanan GR, Zimmerman S, et al. A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the intercontinental childhood ITP study group. J Pediatr2003;143(5):605-608.
3. Donato H, Picon A, Martinez M, et al. Demographic data, natural history, and prognostic factors of idiopathic thrombocytopenic purpura in children: a multicentered study from argentina. Pediatr Blood Cancer2009;52(4):491-496.