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Klinische und pathologische Charakteristika intravaskulärer Lymphome

Clinical and pathological characteristics of intravascular lymphomas

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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).

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.

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  1. Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Straße 1, 30625, Hannover, Deutschland

    L. Abraham, H. Kreipe &  K. Hussein

  2. Institut für Diagnostische und Interventionelle Neuroradiologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    P. Raab

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Abraham, L., Kreipe, H., Raab, P. et al. Klinische und pathologische Charakteristika intravaskulärer Lymphome. Pathologe 39, 242–248 (2018). https://doi.org/10.1007/s00292-018-0427-9

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