Der Pathologe

, Volume 34, Issue 3, pp 225–232 | Cite as

Grauzonenlymphome

Grenzbereiche der Klassifikation aggressiver B-Zell-Lymphome
Schwerpunkt
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Zusammenfassung

Grauzonenlymphome sind lymphatische Tumoren, die aus morphologischen, klinischen oder biologischen Gründen keiner definierten Entität zugeordnet werden können. Sie zeigen in der Regel Eigenschaften, die in zwei sich überlappenden Entitäten zu finden sind oder zwischen diesen liegen. Es kann sich um Tumoren handeln, die in der Grauzone zwischen indolenten und aggressiven Lymphomen zu finden sind oder aber Merkmale aufweisen, die zwischen morphologisch ähnlichen, klinisch und biologisch aber unterschiedlichen Tumoren wie primär mediastinalen großzelligen B-Zell Lymphomen oder primär nodalen diffusen großzelligen B-Zell Lymphomen liegen.

Als eine besondere Kategorie wurde das „B-Zell-Lymphom, unklassifiziert, mit Merkmalen zwischen einem diffusen großzelligen B-Zell-Lymphom und einem Burkitt-Lymphom“ erkannt. Dies beschreibt eine praktische Kategorie, die offenbar verschiedene Entitäten beinhaltet, denen zum Teil ein „Burkitt-ähnlicher“ Aspekt und ein aggressives klinisches Verhalten gemeinsam sind. In genetischer Hinsicht zeigen die Tumoren häufig atypische MYC-Translokationen und zusätzlich komplexe Karyotypalterationen. Ein befriedigendes Therapiekonzept gibt es derzeit nicht.

Schlüsselwörter

Malignes Lymphom Grauzonenlymphom Burkitt-Lymphom Diffuses großzelliges B-Zell-Lymphom Klassifikation 

Abkürzungen

ABC-DLBCL

Diffuses großzelliges B-Zell-Lymphom vom Typ der aktivierten B-Zellen

BL

Burkitt-Lymphom

BCLU-DLBCL/BL

B-Zell-Lymphom, unklassifiziert, mit Merkmalen zwischen einem diffusen großzelligen B-Zell-Lymphom und einem Burkitt-Lymphom

BCLU-DLBCL/HL

B-Zell-Lymphom, unklassifiziert, mit Merkmalen zwischen einem diffusen großzelligen B-Zell-Lymphom und einem Hodgkin-Lymphom

B-CLL

Chronische lymphatische Leukämie vom B-Zell-Typ

DH/TH

Double-hit-/Triple-hit-Lymphom

DLBCL

Diffuses großzelliges B-Zell-Lymphom

FL

Follikuläres Lymphom

GCB-DLBCL

Diffuses großzelliges B-Zell-Lymphom vom Keimzentrumstyp

GEP

Genexpressionsprofil

IGH

Immunglobulin-Schwerketten-Gen

PMBL

Primär mediastinales großzelliges B-Zell-Lymphom

Grey zone lymphomas

Limitations of the classification of aggressive B-cell lymphomas

Abstract

Grey zone lymphomas are lymphatic tumors that cannot be assigned to a defined lymphoma entity due to morphological, clinical or genetic reasons. As a defining criterion they present with features of two overlapping entities or features that are intermediate. Such lymphomas may represent a grey zone in the differentiation between indolent and aggressive lymphomas. Often they may show morphological features of one entity but be more related to another entity with respect to the immunophenotype and/or genetic constitution, such as lymphomas in the grey zone between primary mediastinal large B-cell lymphoma and primary nodal diffuse large B-cell lymphoma. The B-cell lymphoma, unclassified, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma has recently been recognized as a provisional category in the updated WHO 2008 classification of malignant lymphomas. This corresponds to a practical lymphoma category that obviously contains several entities with a Burkitt-like appearance and aggressive clinical behavior. Genetically, tumors in this category are frequently characterized by an atypical MYC translocation and complex karyotypic alterations. As yet, no adequate therapy concept exists.

Keywords

Malignant lymphoma Grey zone lymphoma Burkitt lymphoma Lymphoma, large B-cell, diffuse Classification 

Notes

Interessenkonflikt

Der korrespondierende Autor gibt für sich und seine Koautoren an, dass kein Interessenkonflikt besteht.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Pathologisches InstitutCaritas-KrankenhausBad MergentheimDeutschland
  2. 2.Abteilung für Klinische PathologieRobert-Bosch-Krankenhaus und Dr. Margarete Fischer-Bosch Institut für Klinische PharmakologieStuttgartDeutschland
  3. 3.Pathologisches InstitutUniversität WürzburgWürzburgDeutschland
  4. 4.Abteilung für Klinische PathologieRobert-Bosch-KrankenhausStuttgartDeutschland

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