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Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F22%3A00076163" target="_blank" >RIV/65269705:_____/22:00076163 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216224:14110/22:00125368

  • Result on the web

    <a href="https://www.nature.com/articles/s41375-021-01406-y" target="_blank" >https://www.nature.com/articles/s41375-021-01406-y</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1038/s41375-021-01406-y" target="_blank" >10.1038/s41375-021-01406-y</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis

  • Original language description

    In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level &gt;= 125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p &lt; 0.05) and better overall survival (p &lt; 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels &lt;125 ng/mL.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30205 - Hematology

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2022

  • Confidentiality

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

Data specific for result type

  • Name of the periodical

    Leukemia

  • ISSN

    0887-6924

  • e-ISSN

    1476-5551

  • Volume of the periodical

    36

  • Issue of the periodical within the volume

    2

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    9

  • Pages from-to

    516-524

  • UT code for WoS article

    000698099800004

  • EID of the result in the Scopus database

    2-s2.0-85115206854