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Essential Thrombocythemia in children and adolescents

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Essential Thrombocythemia in children and adolescents Y. Haskel, R. Elhasid, E. Shabad, A. Ballin Background E. T. is an uncommon myeloproliferative disorder ... – PowerPoint PPT presentation

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Title: Essential Thrombocythemia in children and adolescents


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 to
    adults).
  • mortality - 11

5
(No Transcript)
6
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 in children and
    adolescents.

7
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 gt600000/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.

8
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.

9
Results (II)
  • Hematological data at presentation
  • pediatric pts
    adult 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

10
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 or Agrylin.

11
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 and adolescents.
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