Most pediatric patients with essential thrombocythemia show hypersensitivity to erytropoietin in vitro, with rare JAK2 V617F-positive erythroid colonies

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F61989592%3A15110%2F08%3A00006187" target="_blank" >RIV/61989592:15110/08:00006187 - isvavai.cz</a>

Result on the web

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DOI - Digital Object Identifier

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Result language

angličtina

Original language name

Most pediatric patients with essential thrombocythemia show hypersensitivity to erytropoietin in vitro, with rare JAK2 V617F-positive erythroid colonies

Original language description

Essential thrombocythemia (ET), a Philadelphia (Ph) chromosome negative chronic myeloproliferative disorder, is usually a disease of middle age and it is extremely rare in pediatric patients. In this report we studied 12 children diagnosed with ET and one child with thrombocytosis and family history of ET. We failed to detect JAK2 V617F mutation either in peripheral blood leukocytes or in separated platelets and granulocytes. Monoclonal hematopoiesis was noted in only one female patient. Erythroid progenitors of most of the patients displayed hypersensitivity to erythropoietin (Epo) in vitro; Epo-independent erythroid colonies (EECs) were detected in seven patients. Among EECs of three patients we observed rare colonies heterozygous or homozygous for the JAK2 V617F mutation. Our data suggest that childhood ET patients could bear minor JAK2 V617F-positive subclones.

Czech name

Většina dětských pacientů s esenciální trombocytemii s ojedinělými výskytem JAK2 V617F pozitivních erytroidních kolonií vykazuje hypersenzitivitu na erytropoetin v in vitro kulturách

Czech description

Esenciální trombocytemie (ET) je Ph negativní chronické myeloproliferativní onemocnění. Je považováno za onemocnění středního věku a u dětí se vyskytuje velmi vzácně. Tato studie popisuje 12 dětí u kterých byla diagnostikována ET a 1 dítě s trombocytemiía s rodinným zatížením ET.................

Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FD - Oncology and haematology

OECD FORD branch

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Project

<a href="/en/project/NR9471" target="_blank" >NR9471: Myeloproliferative diseases in children</a><br>

Continuities

Z - Vyzkumny zamer (s odkazem do CEZ)

Publication year

2008

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Leukemia Research

ISSN

0145-2126

e-ISSN

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Volume of the periodical

32

Issue of the periodical within the volume

3

Country of publishing house

GB - UNITED KINGDOM

Number of pages

9

Pages from-to

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UT code for WoS article

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EID of the result in the Scopus database

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