Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F18%3A00069092" target="_blank" >RIV/65269705:_____/18:00069092 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/18:00104163

Result on the web

<a href="http://www.bloodjournal.org/content/bloodjournal/131/26/2877.full.pdf?sso-checked=true" target="_blank" >http://www.bloodjournal.org/content/bloodjournal/131/26/2877.full.pdf?sso-checked=true</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1182/blood-2018-03-839753" target="_blank" >10.1182/blood-2018-03-839753</a>

Result language

angličtina

Original language name

Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease

Original language description

Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. RDD, which now belongs to the R group of the 2016 revised histiocytosis classification, is a widely heterogeneous entity with a range of clinical phenotypes occurring in isolation or in association with autoimmune or malignant diseases. Recent studies have found NRAS, KRAS, MAP2K1, and ARAF mutations in lesional tissues, raising the possibility of a clonal origin in some forms of RDD. More than 1000 reports have been published in the English literature; however, there is a lack of consensus regarding approach for the clinical management of RDD. Although in most cases RDD can be observed or treated with local therapies, some patients with refractory or multifocal disease experience morbidity and mortality. Here we provide the first consensus multidisciplinary recommendations for the diagnosis and management of RDD. These recommendations were discussed at the 32nd Histiocyte Society Meeting by an international group of academic clinicians and pathologists with expertise in RDD. We include guidelines for clinical, laboratory, pathologic, and radiographic evaluation of patients with RDD together with treatment recommendations based on clinical experience and review of the literature.

Czech name

—

Czech description

—

Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30205 - Hematology

Project

—

Continuities

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Publication year

2018

Confidentiality

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

Name of the periodical

Blood

ISSN

0006-4971

e-ISSN

—

Volume of the periodical

131

Issue of the periodical within the volume

26

Country of publishing house

US - UNITED STATES

Number of pages

14

Pages from-to

2877-2890

UT code for WoS article

000439123700004

EID of the result in the Scopus database

2-s2.0-85049122426