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Reported as approved
Clinical Standards Group
to the:
Effectiveness Sub Board
This guideline has been approved by the Trust's Clinical Guidelines Assessment Panel as an aid to the diagnosis and
management of relevant patients and clinical circumstances. Not every patient or situation fits neatly into a standard
guideline scenario and the guideline must be interpreted and applied in practice in the light of prevailing clinical
circumstances, the diagnostic and treatment options available and the professional judgement, knowledge and
expertise of relevant clinicians. It is advised that the rationale for any departure from relevant guidance should be
documented in the patient's case notes.
The Trust's guidelines are made publicly available as part of the collective endeavour to continuously improve the
quality of healthcare through sharing medical experience and knowledge. The Trust accepts no responsibility for any
misunderstanding or misapplication of this document .
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Children with ITP should not be admitted to hospital unless they
have ongoing significant bleeding.
Rationale
Childhood ITP is uncommon and causes much (generally unjustified) anxiety regarding
mortality and morbidity. There have been significant differences in investigation and
treatment practice throughout the UK as good quality evidence has been scanty. In
recent years, however, large surveys of practice and outcome as well as a few
randomized trials of different treatment modalities have been conducted. Guidelines on
best practice have been updated as a result, and this guideline draws on the most
recent international guidelines. National audits of practice have been carried out and
results have further informed this guideline.
Definitions
Nomenclature
Newly diagnosed ITP remission occurs before 6 months (50-70%)
1. Pathophysiology
ITP is an acute immune-mediated condition of platelet destruction most commonly
affecting young children. 80-85% remit spontaneously within a few months; 15-20% run
a chronic course of >12 months duration
2. Clinical features
2.1 History
2.2 Examination
Typical features:
Purpura, petechiae and echymoses
Occasionally mucosal bleeding e.g. nosebleeds (and occasionally melaena)
Rarely, macroscopic haematuria
3. Investigations
In typical clinical cases, the only investigations required are a Full Blood Count (FBC),
coagulation screen and Film, which shows normal coagulation screen and
thrombocytopenia (platelet count usually <20x10 9/L) with an otherwise normal film and
counts.
Atypical features on the FBC, which should prompt a review of the diagnosis, include:
Hb <10g/L (in infant <12months); <11g/L (in child >12months)
WBC <5x109/L in child <6yrs; <4x109/L in child >6yrs
Neutrophils <1.5 x109/L
Blast cells on peripheral blood film
4. Management
4.1 Hospital admission: Only patients requiring active treatment or close monitoring
for ongoing bleeding require admission
5.Treatment
Treatment should be based on severity of symptoms not platelet count alone. The goal
of all treatment strategies is to achieve a platelet count that is associated with adequate
haemostasis rather than a normal platelet count.
Treatment can be divided into the following:
Observation only
Used for mild to moderate bleeding
Advise children and parents to exert caution regarding activities associated with
trauma e.g. ski-ing, any contact sport e.g. rugby, boxing.Helmets should be worn
if cycling and if swimming, no diving is recommended in shallow end.
Pharmacological Treatment
Use for severe bleeding and following life-threatening haemorrhage
Raises platelet count faster than no treatment [median time to achieve
platelets over 20x109/L is 1 day for IVIg; 2 days for steroids]
Has no proven effect on the rate of serious or fatal haemorrhages
Has no effect on the incidence of chronic ITP (longer than 12 months)
Is associated with a significant risk of side effects
Tranexamic acid may be prescribed (in consultation with Consultant) for
troublesome persistent symptoms - ensure no haematuria present when using
tranexamic acid
www.ivig.nhs.uk
http://intranet/committees/DTMM/docs/ImmunoglobulinRequestForm.doc
5.3.2.1 Prednisolone
6.Follow-up
2. All children who receive specific treatment for ITP (IVIg or Steroids) should have
documented evidence of life-threatening or severe haemorrhage
3. All children who receive platelet transfusion for ITP should have evidence of life-
threatening haemorrhage
4. All children who receive specific treatment for ITP (IVIg or Steroids) should have daily
FBC monitoring for minimum of 2 days and ongoing monitoring demonstrating recovery
of platelet count >50x109/L
An earlier guideline written by a different author was in use in the Paediatric department
from April 1996 and subsequently updated in 2001 and again in 2012 following a further
review of the published literature and the results of an audit of practice against that
guideline, performed in 1997.
More recent publications have again been reviewed and the guideline modified
accordingly in June 2014. This revised guideline has been circulated for comments by
medical and nursing staff in the Paediatric department and by medical staff in the
Haematology department during the month of May 2014. Comments regarding both
content and style of presentation have been incorporated.
This version has been endorsed by the Clinical Guidelines Assessment Panel.
Grainger JD et al. Changing trends in the UK management of childhood ITP. Arch Dis
Child 2012;97:8-11
Klaasen RJ, Doyle JJ, Krahn MD, Blanchette VS Naglie G. Initial bone marrow aspirate
in childhood idiopathic thrombocytopenic purpura : decision analysis. J Pediatr Hematol
Oncol 2001;23:511-18
Moser AM, Shaler H, Kapelushnik J. Anti-D exerts a very early response in childhood
acute idiopathic thrombocytopenic purpura. Pediatr Hematol Oncol 2002;19:407-11
Introduction
This explains about immune thrombocytopenic purpura (ITP), which is a blood disorder
affecting the platelets. It also explains what to expect when your child is diagnosed with
the condition.
What are platelets?
Platelets are one of the three types of blood cell, along with red and white blood cells.
Platelets are small and sticky and their job is to prevent bruising and stop bleeding after
an injury. Platelets, like red and white blood cells, are formed in the bone marrow. A
rough idea of how many platelets are circulating in the bloodstream (platelet count) can
be made using a sample of blood. The normal platelet count is between is 150 to 400 x
109/l. In most cases of ITP the platelet count is less than 20 x 10 9/l. A low platelet count
is called thrombocytopenia.
A prolonged (over 30 minutes) nosebleed which will not stop despite pinching the
nose
Prolonged gum bleeding
Blood in the poo or urine
Following a heavy blow to the head, particularly if the child is stunned or sickly
Persistent or severe headache
Vomiting or drowsiness
Children on steroids are at a greater risk of a severe form of chickenpox. If your
child has not had chicken pox then contact the hospital If your child is in direct
contact with someone who has chicken pox or who develops chickenpox within 7
days of being with your child.
Is there a UK registry?