Clinical Presentation
ITP has a wide spectrum of presentation in both adults and children—from acute symptoms to incidental diagnosis. Acute ITP is characterized by a sudden onset of symptoms (such as purpura or mucosal bleeding). It is the main presentation in children and is usually self-limiting. Chronic ITP has an insidious onset.1,2 The most common form in adults, it can present many treatment challenges.1
Children
In children, petechiae and bruising may have a sudden onset.3 Children may present (in the ER or to their physician) with platelet counts as low as 5,000/µL and most have counts under 20,000/ µL.3 Intracranial hemorrhage (ICH) is perhaps the most immediate concern for all patients with platelet counts less than 20,000/µL, particularly for those with counts under 10,000/µL.4 Several studies and surveys have suggested a low risk of severe hemorrhage in acute childhood ITP (3% to 6%). The incidence of intracranial hemorrhage (ICH) is lower still—between 0.2% and 1%.5,6
Classification of Clinical Symptoms of Childhood ITP7
| None | No symptoms beyond low platelet count |
| Mild | Bruising and petechiae |
| Occasional minor epistaxis | |
| Very little or no interference with daily living | |
| Moderate | More severe skin manifestations with some mucosal lesions |
| More troublesome epistaxis and menorrhagia | |
| Severe | Bleeding episodes (epistaxis, melena, and/or menorrhagia) requiring hospital admission and/or blood transfusion |
| Serious interference with quality of life |
| Classification system developed in: Bolton-Maggs PHB, Moon I. Assessment of UK practice for management of acute childhood idiopathic thrombocytopenic purpura against published guidelines. Lancet. 1997;350:620-623. |
Adults
In contrast to the typically acute onset of childhood ITP, the onset of ITP in adults is often insidious. Adults are commonly asymptomatic with only mild bruising.1 Many are diagnosed incidentally from a routine blood count.8 Bleeding is usually seen only in severe cases (platelet count < 30,000/ µL).1 Adult patients presenting with symptomatic thrombocytopenia are often treated immediately with glucocorticoids, making a true assessment of the clinical course of untreated disease difficult.9 A study of patients with borderline thrombocytopenia (100-150,000/µL) found that within 6 months 6.9% of them developed ITP with platelet counts consistently below 100,000/µL).10
References
1. British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol. 2003;120(4):574-596.
2. Tierney LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis & Treatment. 43rd ed. New York, NY: Lange Medical Books/McGraw-Hill; 2004:498-499.
3. Buchanan GR. Bleeding signs in children with idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol. 2003;25(Suppl 1):S42-S46.
4. Bussel J. Treatment of immune thrombocytopenic purpura in adults. Semin Hematol. 2006;43(3 Suppl 5):S3-10; discussion S18-19.
5. Tarantino MD, Bolton-Maggs PHB. Update on the management of immune thrombocytopenic purpura in children. Curr Opin Hematol. 2007;14: 526-534.
6. Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med. 2002;346(13):995-1008.
7. Bolton-Maggs PHB, Moon I. Assessment of UK practice for management of acute childhood idiopathic thrombocytopenic purpura against published guidelines. Lancet. 1997;350:620-623.
8. Neylon AJ, Saunders PWG, Howard MR, Proctor SJ, Taylor PRA. Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients. Br J Haematol. 2003;122(6):966-974.
9. George JN, Woolf SH, Raskob GE, et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood. 1996;88(1):3-40.
10. Stasi R, Amadori S, Osborn J, Newland AC, Provan D. Long-term outcome of otherwise healthy individuals with incidentally discovered borderline thrombocytopenia. PLoS Med. 2006;3(3):e24.
