Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F16%3A10335791" target="_blank" >RIV/00064203:_____/16:10335791 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/16:00092612 RIV/00216208:11130/16:10335791 RIV/65269705:_____/16:00066359

Result on the web

<a href="http://dx.doi.org/10.3324/haematol.2016.147116" target="_blank" >http://dx.doi.org/10.3324/haematol.2016.147116</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3324/haematol.2016.147116" target="_blank" >10.3324/haematol.2016.147116</a>

Result language

angličtina

Original language name

Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents

Original language description

Children and adolescents with pre-existing conditions such as DNA repair defects or other primary immunodeficiencies have an increased risk of non-Hodgkin lymphoma. However, large-scale data on patients with non-Hodgkin lymphoma and their entire spectrum of pre-existing conditions are scarce. A retrospective multinational study was conducted by means of questionnaires sent out to the national study groups or centers, by the two largest consortia in childhood non-Hodgkin lymphoma, the European Intergroup for Childhood non-Hodgkin Lymphoma, and the international Berlin-Frankfurt-Munster Study Group. The study identified 213 patients with non-Hodgkin lymphoma and a pre-existing condition. Four subcategories were established: a) cancer predisposition syndromes (n=124, 58%); b) primary immunodeficiencies not further specified (n=27, 13%); c) genetic diseases with no increased cancer risk (n=40, 19%); and d) non-classifiable conditions (n=22, 10%). Seventy-nine of 124 (64%) cancer predispositions were reported in groups with more than 20 patients: ataxia telangiectasia (n=32), Nijmegen breakage syndrome (n=26), constitutional mismatch repair deficiency (n=21). For the 151 patients with a known cancer risk, 5-year event-free survival and overall survival rates were 40%+/- 4% and 51%+/- 4%, respectively. Five-year cumulative incidences of progression/relapse and treatment-related death as a first event were 22%+/- 4% and 24%+/- 4%, respectively. Ten-year incidence of second malignancy was 24%+/- 5% and 7-year overall survival of the 21 patients with a second malignancy was 41%+/- 11%. Patients with non-Hodgkin lymphoma and pre-existing conditions have an inferior survival rate with a large proportion of therapy-related deaths compared to patients with non-Hodgkin lymphoma and no pre-existing conditions. They may require special vigilance when receiving standard or modified/reduced-intensity chemotherapy or when undergoing allogeneic stem cell transplantation.

Czech name

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Czech description

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Type

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

CEP classification

FD - Oncology and haematology

OECD FORD branch

—

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2016

Confidentiality

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

Name of the periodical

Haematologica

ISSN

0390-6078

e-ISSN

—

Volume of the periodical

101

Issue of the periodical within the volume

12

Country of publishing house

IT - ITALY

Number of pages

11

Pages from-to

1581-1591

UT code for WoS article

000392584400018

EID of the result in the Scopus database

2-s2.0-85002412781