[Retrospective analysis of the clinical course of 12 children given the diagnosis essential thrombocythemia].

Abstract:

BACKGROUND:Essential Thrombocythemia (ET) is an acquired myeloproliferative disorder (MPD) characterized by excessive production of platelets. The disorder is usually affecting adults and is rarely diagnosed in children. PATIENTS AND METHODS:In this retrospective study we describe 12 children aged 5-16 years in whom ET was presumed. RESULTS:Median follow-up was 59 months (range 10-72). At diagnosis 7 patients had clinical symptoms (syncope, poor concentration, fatigue, abdominal pain and mild bleeding), 5 patients were diagnosed accidentally (operation, allergy, enuresis, pneumonia, routine examination). Median platelet count at diagnosis was 1 325 x 10 (9)/L (range 600-3 050). In 11 cases bone marrow morphology was consistent with ET, one patient had chronic idiopathic myelofibrosis. Cytogenetics were normal in all studied cases. Within 6 months after the initial presentation one patient who was diagnosed accidentally developed thrombosis, another patient had mild bleeding. 8 patients were treated with acetylsalicylic acid (in addition, 1 patient received hydroxyurea, 2 patients received anagrelide). On last follow-up all patients were alive, none had developed leukemia. 5 patients experienced hematological remission. 2 of these children had not received any therapy. CONCLUSIONS:Many patients had symptoms attributable to ET. The clinical course is heterogeneous with complete normalization of platelets in the absence of cytoreductive therapy in some children. Due to the low incidence of ET in children indications for therapy are unclear and can only be deduced from findings obtained from studies in adults.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Kratz CP,Abd El-Monheim A,Manke EM,Rister M,Rogge T,Niemeyer CM

doi

10.1055/s-2004-832356

keywords:

subject

Has Abstract

pub_date

2004-11-01 00:00:00

pages

349-55

issue

6

eissn

0300-8630

issn

1439-3824

journal_volume

216

pub_type

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