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Journal of Laboratory Medicine

Official Journal of the German Society of Clinical Chemistry and Laboratory Medicine

Editor-in-Chief: Schuff-Werner, Peter

Ed. by Ahmad-Nejad, Parviz / Bidlingmaier, Martin / Bietenbeck, Andreas / Conrad, Karsten / Findeisen, Peter / Fraunberger, Peter / Ghebremedhin, Beniam / Holdenrieder, Stefan / Kiehntopf, Michael / Klein, Hanns-Georg / Kohse, Klaus P. / Kratzsch, Jürgen / Luppa, Peter B. / Meyer, Alexander von / Nebe, Carl Thomas / Orth, Matthias / Röhrig-Herzog, Gabriele / Sack, Ulrich / Steimer, Werner / Weber, Thomas / Wieland, Eberhard / Winter, Christof / Zettl, Uwe K.


IMPACT FACTOR 2018: 0.389

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2567-9449
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Volume 39, Issue 5

Issues

Differenzialdiagnose BCR-ABL1-negativer myeloproliferativer Neoplasien

Differential diagnosis of BCR-ABL1-negative myeloproliferative neoplasms

Hans Michael Kvasnicka
  • Corresponding author
  • Senckenberg Institut für Pathologie, Universität Frankfurt, Theodor-Stern-Kai 7, 60590 Frankfurt am Main, Deutschland
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  • De Gruyter OnlineGoogle Scholar
/ Martin Grießhammer
  • Klinik für Hämatologie, Onkologie und Palliativmedizin, Mühlenkreiskliniken, Johannes Wesling Klinikum Minden, Minden, Deutschland
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2015-09-25 | DOI: https://doi.org/10.1515/labmed-2015-0079

Zusammenfassung:

Die myeloproliferativen Neoplasien (MPN) gehen auf klonale Stammzellveränderungen zurück und zeigen eine unterschiedliche Knochenmarksmorphologie. Die Differenzialdiagnose zwischen den drei BCR-ABL1-negativen Entitäten essentielle Thrombozythämie, Polyzythaemia vera und primäre Myelofibrose ist häufig schwierig und sollte nach aktuellen Erkenntnissen auf den Kriterien der WHO basieren. Die Entdeckung neuer molekulare Marker wie Calreticulin (CALR) erlaubt eine bessere Abgrenzung einer MPN von reaktiven Veränderungen, wobei der alleinige Mutationsnachweis für die differenzialdiagnostische Abgrenzung der einzelnen Subtypen aber meist nicht ausreichend ist. Insofern ist insbesondere in frühen Stadien der MPN, die oftmals mit einer anhaltenden Thrombozytose einhergehen, eine gemeinsame Betrachtung hämatologischer, molekulargenetischer, zytologischer und vor allem histomorphologischer Befunde notwendig.

Abstract:

Myeloproliferative neoplasms (MPN) are clonal stem cell disorders presenting with a broad spectrum of changes in bone marrow morphology, clinical findings, and overall outcome. The differential diagnosis between the three classical BCR-ABL1-negative subtypes essential thrombocythemia, polycythemia vera, and primary myelofibrosis may be difficult and should be based on the current diagnostic criteria proposed by the WHO. In this regard the discovery of new molecular markers as calreticulin (CALR) enables a better discrimination of reactive changes, however, mutation screening alone does not increase diagnostic specificity and subtyping of MPN. Therefore, in early stages of MPN which may present with a sustained thrombocytosis, a synoptical diagnostic approach with inclusion of hematological, molecular, cytological, and in particular histomorphological findings is essential.

Reviewed publication

NebeC.T.

Schlüsselwörter:: myeloproliferative Neoplasien; essentielle Thrombozythämie (ET); primäre Myelofibrose (PMF); Polyzythaemia vera (PV); Histopathologie; klinische Befunde; WHO-Klassifikation; Differenzialdiagnose

Keywords:: clinical findings; differential diagnosis.; essential thrombocythemia (ET); histopathology; primary myelofibrosis (PMF); myeloproliferative neoplasms; polycythemia vera (PV); WHO-classification

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About the article

Korrespondenz: Prof. Dr. Hans Michael Kvasnicka, Senckenberg Institut für Pathologie, Universität Frankfurt, Theodor-Stern-Kai 7, 60590 Frankfurt am Main, Deutschland, Tel.: +49-69-6301-4900, Fax: +49-69-6301-3903, E-Mail:


Received: 2015-08-21

Accepted: 2015-09-07

Published Online: 2015-09-25

Published in Print: 2015-10-01


Citation Information: LaboratoriumsMedizin, Volume 39, Issue 5, Pages 301–310, ISSN (Online) 1439-0477, ISSN (Print) 0342-3026, DOI: https://doi.org/10.1515/labmed-2015-0079.

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