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[Primary thrombocythemia in childhood].

B Flapper, C Van Oostrom, G de Vaan

    Tijdschrift Voor Kindergeneeskunde
    |June 1, 1983
    PubMed
    Summary

    Primary thrombocythemia, a rare childhood myeloproliferative disorder, involves high platelet counts. This study highlights two pediatric cases, suggesting it may occur without significant complications in younger patients.

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    Results of splenectomy performed on a group of 91 children.

    European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie·1995

    Area of Science:

    • Hematology
    • Pediatric Oncology

    Background:

    • Primary thrombocythemia is a myeloproliferative neoplasm defined by elevated platelet counts (>1000 x 10^9/L).
    • While common in adults, it is rarely reported in pediatric populations.

    Observation:

    • Two pediatric cases of primary thrombocythemia are presented: a 13.5-year-old boy with incidental findings and a 12-year-old girl with thrombotic and bleeding symptoms post-splenectomy.
    • Both patients exhibited significantly elevated platelet counts (2167 x 10^9/L and 5083 x 10^9/L, respectively), splenomegaly, and abnormal peripheral blood/bone marrow findings.
    • The girl experienced microvascular disturbances and cerebrovascular symptoms attributed to high platelet counts.

    Findings:

    • Treatment with melphalan, aspirin, and dipyridamole led to symptom resolution in both patients as platelet counts decreased.
    • Abnormal platelet function, assessed via aggregation studies, was noted in both cases.
    • The study suggests that primary thrombocythemia in children may have a less complicated course compared to adults.

    Implications:

    • Pediatric primary thrombocythemia might be manageable with a lower risk of severe complications.
    • Anti-aggregating agents could be a primary treatment consideration for children with this condition.
    • Further research into the long-term outcomes and optimal management strategies for pediatric primary thrombocythemia is warranted.

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