Elsevier

The Journal of Pediatrics

Volume 143, Issue 3, September 2003, Pages 302-307
The Journal of Pediatrics

Review article
Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: a prospective nordic study of an unselected cohort

https://doi.org/10.1067/S0022-3476(03)00245-2Get rights and content

Abstract

Objective

To determine the duration of the risk period with platelet counts <20×109/L and the frequency of bleeding episodes in unselected children with idiopathic thrombocytopenic purpura (ITP).

Study design

We established a registry for patients with newly diagnosed ITP in the five Nordic countries, enrolling children aged 0 to 14 years with platelet counts <30×109/L. Treatment centers prospectively reported presenting features, management details, and disease-related events during the first six months after diagnosis.

Results

At presentation (n = 501), more than half of the children had a platelet count <10×109/L, but only 15 (3.0%) had a hemorrhage requiring blood transfusion. During follow-up of 409 patients, thrombocytopenia resolved uneventfully in 277. A risk period was present in 376 cases. Among 283 with self-limiting ITP, 26 were at risk >1 month and 25 had 30 events. Among 93 patients with chronic ITP, 73 were at risk >1 month and 44 had 111 events. Events occurred with an average frequency of 0.39 per month at risk. Life-threatening hemorrhages did not occur in the first six months after diagnosis.

Conclusion

Most children with ITP are at risk for serious bleeding for less than one month. Continuing severe thrombocytopenia is associated with little morbidity, bleeding episodes being infrequent and very rarely serious.

Section snippets

Methods

All pediatric departments in the five Nordic countries were invited to participate in a prospective study of patients with newly diagnosed ITP in the period from January 1, 1998 to December 31, 1999 with an expected accrual of approximately 500 cases. At each center, a specified pediatrician was responsible for mailing registration forms to a national coordinator after initial management and for following the children for at least six months and then reporting the course. Registration was

Results

Ninety-eight institutions participated and enrolled 386 newly diagnosed cases in the study period: 167 in 1998 (study not open in Norway) and 219 in 1999. Registration was continued in some centers throughout 2000, bringing an additional 116 children to the study and increasing accrual to a total of 502 children. The initial diagnosis was revised later in the course in one child with myelodysplastic syndrome. Thus, the case series comprises 501 children: 161 from Sweden, 152 from Finland, 109

Discussion

In this prospective study of an unselected cohort of 501 children with ITP causing platelet counts <30×109/L, we found that the bleeding tendency generally is mild, that the morbidity is limited, and that the risk of serious bleeding is small. Most children had profound thrombocytopenia, but significant mucosal bleeding was rare. During follow-up, bleeding episodes occurred at a surprisingly low frequency and usually were trivial. Life-threatening bleeding did not occur in the first six months

Acknowledgements

We thank all the members of the national study groups who entered patients into the study and carefully reported the necessary data.

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    However, mucosal bleeding and a younger age are also known as characteristics associated with a lower frequency of developing chronic ITP. The observed rate of chronic ITP after 1 year of 11% is substantially lower than historical data,1-5 and lower compared with the 28% reported by the Intercontinental Cooperative ITP Study Group.6 In accordance with the literature,24 also in our trial, a high recovery rate between 6 and 12 months after diagnosis was observed: 54% of children with a platelet count lower than 150 × 109/L and 45% of children with a platelet count lower than 100 × 109/L at 6 months showed complete response (platelet count >100 × 109/L) at 12 months, partly explaining the lower rate of chronic ITP observed in our trial when applying the current definition of chronic ITP.

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This study was carried out as a NOPHO study without any financial support, and no author was influenced by relationships to other organizations.

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