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Cultures of myeloid progenitor cells in pediatric essential thrombocythemia

Leukemia volume 16, pages 1876–1877 (2002)Cite this article

Essential thrombocythemia (ET) is a chronic myeloproliferative disorder (CMPD) characterized by hyperproliferation of megakaryocytes and persistent thrombocytosis. Thrombosis, bleeding and microvascular occlusive symptoms represent the major cause of morbidity and mortality in ET patients. Thrombocytosis defined as a platelet count higher than 500 × 109/l is a frequent hematological finding in childhood, occuring in 3 to 13% of pediatric patients.1 Secondary or reactive causes such as infection, trauma or surgery, iron deficiency, hypoesplenism, nonhematologic malignancies or rebound from chemotherapy, represent the etiology of most cases. In contrast, ET is extremely rare at the pediatric age,2 although at present, with the advent of automated platelet counting, it is being diagnosed with increasing frequency at a younger age. To date no more than 50 pediatric cases of ET have been reported.1,2,3 Familial occurrence is observed in 40–55% of all childhood cases with primary thrombocytosis, and these patients characteristically show neither thrombotic manifestations nor malignant or myelofibrotic transformation during their clinical follow-up.2 ET is currently diagnosed through exclusion of non-ET CMPD and conditions associated with reactive thrombocytosis by criteria set by the Polycythemia Vera Study Group (PVSG).4 A diagnosis of ET in the individual pediatric patient is a matter of great significance given the known prognostic (thrombohemorrhagic complications, and clonal evolution) and therapeutic implications of this CMPD.

Hemopoietic progenitors have attracted attention because some adult patients with ET and other CMPD show endogenous growth.5 The characteristic pattern of endogenous growth is based on the ability of colony formation in the absence of a stimulating agent in the culture medium. This phenomenon is not observed in either normal subjects or in RT.5,6

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Acknowledgements

This work was supported by the grant FIS 01/1424 from the ‘Ministerio de Sanidad y Consumo’. Spain. The excellent technical assistance of RM Vilà is gratefully acknowledged.

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Authors and Affiliations

  1. Laboratori de Citologia Hematològica, Hospital del Mar, Barcelona, Spain

    L Florensa & L Zamora

  2. Servei d'Hematologia Clínica, Hospital del Mar, Barcelona, Spain

    C Besses

  3. Hospital Vall d'Hebron, Barcelona, Spain

    JJ Ortega & P Bastida

  4. Hospital Sant Joan de Deu, Barcelona, Spain

    T Toll

  5. Hospital Miguel Servet, Zaragoza, Spain

    P Mayayo

  6. Laboratori de Citogenètica i Biologia Molecular, Hospital del Mar, Barcelona, Spain

    L Zamora, B Espinet & F Solé

  7. Departament de Patologia, Hospital del Mar, Barcelona, Spain

    S Serrano

  8. Escola de Citologia Hematològica Soledad Woessner-IMAS, Barcelona, Spain

    L Florensa, F Solé & S Woessner

  9. Grup de Recerca Hematològica, Barcelona, Spain

    L Florensa, C Besses, F Solé & S Serrano

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  1. L Florensa
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Florensa, L., Zamora, L., Besses, C. et al. Cultures of myeloid progenitor cells in pediatric essential thrombocythemia. Leukemia 16, 1876–1877 (2002). https://doi.org/10.1038/sj.leu.2402574

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