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Essential Thrombocythemia in children and adolescents

Essential Thrombocythemia in children and adolescents. Y. Haskel, R. Elhasid, E. Shabad, A. Ballin. Background. E. T. is an uncommon myeloproliferative disorder, characterized, mainly, by increased number of platelets and tendency to clot formation and / or bleeding.

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Essential Thrombocythemia in children and adolescents

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  1. Essential Thrombocythemia inchildren and adolescents Y. Haskel, R. Elhasid, E. Shabad, A. Ballin

  2. Background • E. T. is an uncommon myeloproliferative disorder, characterized, mainly, by increased number of platelets and tendency to clot formation and / or bleeding. • E. T. may terminate in AML or pure red cell aplasia. • Thromboembolic complications may be fatal. • E. T. is rare in children and adolescents. • High / low (?) risk of thrombosis in young adults.

  3. Background (contin.) • The clinical picture of pediatric E. T.: • - asymptomatic (lower PLT) • - headache, bleeding, Thrombotic events. • - Physical exam: splenomegaly, hepatomegaly • - Familial thrombocythemia - lower PLT counts, no thrombotic events and no malignancies (auto-dom). • -

  4. Background (contin.) • Thrombopoietin level - normal (increased sensitivity in vitro ?). • Cytogenetic studies - normal • Treatment: aspirin, Hydrea, Anagrelide (!), IFN alfa • 2/36 (6%) patients developed AML (similar toadults). • mortality - 11%

  5. The aim of the study • To assess the incidence of E.T. in the pediatric population in Israel. • To describe the clinical picture and the natural history of the disease inchildren and adolescents.

  6. :Methods and patients • Retrospective review of all charts of pediatric patients (to age 18 y’) in Israel, diagnosed to suffer from E.T. between Jan. 1990 and Dec. 2001. • E. T. - Platelet number >600000/mL, normal RBC mass, no Philadelphia chromosome and absence of any etiology for secondary thrombocytosis. • Demographic and clinical data were compared between the pediatric patients and randomly chosen adults with E. T.

  7. Results (I): • Four children and adolescents were diagnosed to have E. T. • Two boys and two girls • Median age: 11 y’ (7-15 y’) • The incidence of pediatric E. T. in Israel is approximately 1 : 500,000 • Median follow-up: 5.5 years. • No death / serious hematological sequel occurred.

  8. Results (II) • Hematological data at presentation • pediatric ptsadult pts • platelet, med. 1754 (1038-4500) 735 (573-1249) • MPV, median 8.7 (8.1-8.8) 9.9 (8.6-10.1) • Hgb, median 12.7 (12.4-15.7) 13 (12.6-14.4) • leukocytes 10600 (7200-14300) 9350 (7820-10840) • normal aggregation of platelets • collagen 4 4 • adrenalin 0 0 • ADP 1 2

  9. Results (III) • All pediatric E. T. patients were treated with aspirin and Hydrea or Agrylin. • All pediatric patients are doing well. • Two of them need no chemotherapy now. • The adults patients with E. T. are dependent on the treatment. All suffer from side effects of Hydrea orAgrylin.

  10. Summary • 1) E. T. is a rare disease in the pediatric population. • 2) Unlike adult patients with E. T., children and adolescents have a mild course of the disease with no complications or any hematological disorder. • 3) Further investigation is warranted to better characterize E. T. in children andadolescents.

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