Immune thrombocytopenia (ITP) in children: Management of chronic disease
- James B Bussel, MD
James B Bussel, MD
- Professor of Pediatrics, Pediatric Hematology
- Weill Medical College of Cornell University
Immune thrombocytopenia (ITP) of childhood is characterized by isolated thrombocytopenia (platelet count <100,000/microL, with normal white blood cell count and hemoglobin). The cause of ITP remains unknown in most cases, but it can be triggered by a preceding viral infection [1-4]. ITP was previously known as idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura. The current term Immune ThrombocytoPenia preserves the widely-recognized acronym "ITP" and acknowledges the immune-mediated mechanism of the disorder, while allowing that patients may have little or no signs of purpura or bleeding .
ITP in children often resolves spontaneously within three months. Up to 20 percent of affected children will go on to have chronic ITP, which is defined as thrombocytopenia for more than 12 months since presentation [5-7]. (See "Immune thrombocytopenia (ITP) in children: Initial management", section on 'Disease course'.)
The treatment and prognosis of chronic ITP and chronic refractory in children will be reviewed here . The epidemiology, diagnosis, and initial management of ITP in children are discussed separately. (See "Immune thrombocytopenia (ITP) in children: Clinical features and diagnosis" and "Immune thrombocytopenia (ITP) in children: Initial management".)
Approximately 10 to 20 percent of children who present with immune thrombocytopenia (ITP) will develop chronic ITP, defined as thrombocytopenia (platelet count <100,000/microL) continuing beyond 12 months from the time of presentation [9,10]. Risk factors for developing chronic ITP include older age, less severe thrombocytopenia at the initial diagnosis, insidious onset of symptoms, and lack of preceding infection or vaccination prior to development of ITP.(See "Immune thrombocytopenia (ITP) in children: Initial management", section on 'Disease course'.)
At the initial presentation of a child with symptoms suggestive of immune thrombocytopenia (ITP), a basic evaluation should be performed to exclude other causes of thrombocytopenia, including a focused history and physical examination, and laboratory testing consisting of a complete blood count (CBC) with a reticulocyte count, differential white blood cell count and red blood cell indices, and direct antiglobulin test (DAT, also known as a direct Coombs test) (table 1). Children with lymphadenopathy, splenomegaly, systemic symptoms, or any abnormalities in the CBC or peripheral blood smear, should promptly undergo further investigation to exclude other causes of thrombocytopenia. This initial diagnostic evaluation is discussed in detail in a separate topic review. (See "Immune thrombocytopenia (ITP) in children: Clinical features and diagnosis", section on 'Diagnosis'.)
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- Supportive care
- Pharmacologic therapy
- - First-line therapies
- - Second-line therapies
- Thrombopoietin receptor agonists
- Adjunctive therapies
- Spontaneous remission
- Bleeding risk
- CHRONIC REFRACTORY ITP
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS