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Der Pathologe

, Volume 39, Issue 3, pp 242–248 | Cite as

Klinische und pathologische Charakteristika intravaskulärer Lymphome

  • L. Abraham
  • H. Kreipe
  • P. Raab
  • K. Hussein
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Zusammenfassung

Intravaskuläre B‑Zell-Lymphome (IVL) sind seltene Neoplasien, die in jedem Alter auftreten können (Durchschnittsalter ca. 62–63 Jahre). Etwa die Hälfte der Fälle ist mit dem Epstein-Barr-Virus assoziiert. Die häufigsten Manifestationen finden sich im Gehirn, in der Haut und im Knochenmark. Die Diagnostik wird dadurch erschwert, dass die klinische Präsentation und die Laborwerte unspezifisch sind und die FACS(fluorescence-activated cell sorting)- und Klonalitätsanalysen aus dem peripheren Blut sowie die radiologischen Befunde nicht diagnostisch sein können. Die sensitivste und spezifischste diagnostische Methode ist die histopathologische und immunhistochemische Evaluation einer Gewebebiopsie. Wegen der Seltenheit dieser Krankheit gibt es nur wenige Erkenntnisse zu Therapie und Prognose, wobei die Therapie sich weitgehend an aggressiven Nicht-IVL-Lymphomen orientiert. Die Prognose ist schlecht (medianes Überleben nach Diagnose etwa ein Jahr).

Schlüsselwörter

Intravaskuläre Lymphome Histologie Immunhistochemie Neuroradiologie 

Clinical and pathological characteristics of intravascular lymphomas

Abstract

Intravascular B‑cell lymphomas (IVL) are rare neoplasms that can manifest at any age (mean age ~62–63 years). About half of the cases are associated with Epstein-Barr virus. The most common sites of manifestation are the brain, skin, and bone marrow. The diagnosis is difficult due to unspecific clinical presentation and laboratory changes. FACS (fluorescence-activated cell sorting) and clonality analysis from peripheral blood and radiological findings are often not diagnostic. The most sensitive and most specific diagnostic method is the histopathological and immunohistochemical evaluation of a tissue biopsy. Because of the rarity of this disease, little is known about therapy and prognosis, whereby therapy is mainly similar to non-IVL lymphomas. The prognosis is poor; median survival after diagnosis is approximately one year.

Keywords

Intravascular lymphoma Histology Immunohistochemistry Neuroradiology 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

L. Abraham, H. Kreipe, P. Raab und K. Hussein geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Institut für PathologieMedizinische Hochschule HannoverHannoverDeutschland
  2. 2.Institut für Diagnostische und Interventionelle NeuroradiologieMedizinische Hochschule HannoverHannoverDeutschland

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