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WHO-Klassifikation 2022 chronischer myeloproliferativer Erkrankungen

WHO classification 2022 of chronic myeloproliferative neoplasms

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Zusammenfassung

Hintergrund

Menschliche Tumorerkrankungen verschiedener Organsysteme werden von der Weltgesundheitsorganisation (WHO) im Rahmen der 5. Ausgabe der Klassifikation in einer einheitlichen relationalen Datenbank hierarchisch katalogisiert. Die Klassifikation 2022 der hämatologischen und lymphatischen Tumoren liegt jetzt vor und wurde in 2 Vorabpublikationen in der Zeitschrift Leukemia vorgestellt. Die finale Version wird wie üblich in einem „Blauen Buch“ veröffentlicht und ist bereits als „Beta-Version“ online verfügbar.

Ergebnisse

Im vorliegenden Artikel wird das neue WHO-Klassifikationsschema für myeloproliferative und verwandte Neoplasien zusammengefasst. Es wird ein Überblick über die Änderungen gegenüber der vorherigen Ausgabe gegeben sowie über die Daten und Überlegungen, die diesen Änderungen zugrunde liegen. Die Definition und Diagnose von Krankheitsentitäten basiert weiterhin auf klinisch-pathologischen Parametern und wird ergänzt durch therapeutisch und/oder prognostisch verwertbare Biomarker. Während die genetischen Befunde für die Definition der Entitäten zunehmend im Vordergrund stehen, ist die Klassifikation bestrebt, die praktische weltweite Anwendbarkeit im Auge zu behalten. Das Ergebnis ist eine verbesserte, zeitgemäße, evidenzbasierte Klassifikation von myeloischen und histiozytären/dendritischen Neoplasien, die in der Molekularbiologie verwurzelt ist und einen organisatorischen Aufbau aufweist, der eine künftige Erweiterung ermöglicht, wenn neue Erkenntnisse in künftige Ausgaben einfließen.

Abstract

Background

Human neoplasms of various organ systems are being hierarchically cataloged by the World Health Organization (WHO) in a unified relational database as part of the 5th edition of the classification. The 2022 classification of hematologic and lymphoid tumors is now available and was presented in preliminary form in two publications in the journal Leukemia. The final version will be soon published in a “WHO Blue Book” as usual and is currently available online in beta format.

Results

This article summarizes the new WHO classification scheme for myeloproliferative and related neoplasms. Changes from the previous edition and the data and considerations underlying these changes are reviewed. Both definition and diagnosis continue to be based on clinicopathologic parameters and are supplemented by therapeutically and/or prognostically useful biomarkers. While genetic findings are paramount in defining entities, the classification strives to keep practical global applicability in mind. The result is an improved, contemporary, evidence-based classification of myeloid and histiocytic/dendritic neoplasms that is rooted in molecular biology and is organized in a manner that allows for expansion as new evidence is incorporated into future editions.

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Author information

Authors and Affiliations

  1. Abteilung Hämatologie und Internistische Onkologie, Klinik für Innere Medizin II, Universitätsklinikum Jena, Am Klinikum 1, 07747, Jena, Deutschland

    Andreas Hochhaus

  2. Abteilung für Klinische Pathologie, Robert-Bosch-Krankenhaus, Stuttgart, Deutschland

    German Ott

  3. Dr. Margarete Fischer-Bosch-Institut für Klinische Pharmakologie, Stuttgart, Deutschland

    German Ott

  4. Institut für Humangenetik, Universitätsklinikum Ulm, Ulm, Deutschland

    Reiner Siebert

Authors
  1. Andreas Hochhaus
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  2. German Ott
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  3. Reiner Siebert
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Corresponding author

Correspondence to Andreas Hochhaus.

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Interessenkonflikt

A. Hochhaus erhielt institutionelle Forschungsförderung von Novartis, BMS, Pfizer und Incyte. G. Ott und R. Siebert geben an, dass kein Interessenkonflikt besteht.

Additional information

Der vorliegende Beitrag basiert auf „Khoury JD, Solary E, Abla O et al (2022) The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia 36:1703–1719.

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Hochhaus, A., Ott, G. & Siebert, R. WHO-Klassifikation 2022 chronischer myeloproliferativer Erkrankungen. Onkologie 29, 287–295 (2023). https://doi.org/10.1007/s00761-023-01308-x

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  • DOI: https://doi.org/10.1007/s00761-023-01308-x

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