Zusammenfassung
Das lymphozytenprädominante Hodgkin-Lymphom (LPHL) ist ein seltenes Lymphom der B-Zell-Reihe, das sich in seiner Pathologie sowie in seinen klinischen Merkmalen deutlich von anderen Subtypen des Hodgkin-Lymphoms (HL) unterscheidet. Immunphänotypisch unterscheiden sich die lymphozytischen und histiozytischen malignen Zellen (CD20+, CD15−, CD30−) grundlegend von den Tumorzellen des klassischen HL. Patienten mit LPHL sind häufig junge Männer mit indolenten und asymptomatischen Lymphknotenschwellungen peripherer Lymphknotenstationen. Trotz sehr guter Ansprechraten auf eine initiale Therapie werden Spätrezidive z. T. Jahrzehnte nach Therapie beobachtet. Im Stadium IA gilt eine alleinige Involved-Field-Radiotherapie (IF-RT) mit 30 Gy als Behandlungsstandard. Da der Einsatz des monoklonalen Antikörpers Rituximab bei LPHL-Rezidiven vielversprechende Remissionen gezeigt hat, wurde Rituximab kürzlich im Rahmen einer prospektiven Beobachtungsstudie der German Hodgkin Study Group (GHSG) eingesetzt. Für Patienten in intermediären und fortgeschrittenen Stadien sollte eine stadienadaptierte Behandlung gemäß Protokollen für das klassische HL erfolgen.
Abstract
Lymphocyte-predominant Hodgkin’s lymphoma (LPHL) is a rare type of B-cell lymphoma. It is different from the other subtypes of Hodgkin’s lymphoma (HL) because of its pathology and clinical course. Differences are also seen in the immunophenotype of the malignant lymphocytic and histiocytic cells (CD20+, CD15−, CD30−) which differ from the tumor cells in classic HL. Patients are mostly young men with indolent and asymptomatic involvement of peripheral lymph nodes. Despite excellent treatment options in patients with early favorable stages, late relapses are seen over decades after therapy. In stage IA involved-field radiotherapy (IFRT) with 30 Gy is recommended as treatment standard. Recently promising results have been reported by using the chimeric antibody rituximab in the treatment of relapsed patients. Therefore, rituximab was applied in an observational study of the German Hodgkin Study Group (GHSG). Patients in early unfavorable and advanced stages should be treated according to the treatment protocols for classic HL.
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Kriz, J., Müller, RP., Engert, A. et al. Lymphozytenprädominantes Hodgkin-Lymphom. Onkologe 16, 41–47 (2010). https://doi.org/10.1007/s00761-009-1770-5
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DOI: https://doi.org/10.1007/s00761-009-1770-5
Schlüsselwörter
- Lymphozytenprädominantes Hodgkin-Lymphom
- Hodgkin-Lymphom
- Involved-Field-Radiotherapie
- Stadium IA
- Rituximab