Was ist wichtig bei Diagnose und Therapie des nodulär Lymphozyten-prädominanten Hodgkin-Lymphoms?
摘要
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity with an incidence of 0.1–0.2/100,000/year. The pathophysiology and clinical presentation of NLPHL differ from those of classic Hodgkin lymphoma.
ObjectiveThis article aims to provide an overview of the biological and histological characteristics of NLPHL as well as current treatment strategies.
Materials and methodsA selective literature review was performed.
ResultsNodular lymphocyte-predominant Hodgkin lymphoma represents a distinct lymphoma entity. Disease biology and pathology as well as surface markers differ from those of classic Hodgkin lymphoma. These differences—as in the case of CD20—can impact treatment decisions. Patients with stage IA NLPHL without risk factors are treated with radiotherapy alone. For all other early and intermediate stages, very good results are obtained with approaches that are also applied in classic Hodgkin lymphoma. Advanced NLPHL is treated with either intensive BEACOPP-based chemotherapy (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) or with protocols that are frequently used in the management of B‑cell non-Hodgkin lymphomas (e.g., R‑CHOP; rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone). In the case of NLPHL recurrence, only a minority of patients require high-dose chemotherapy and autologous stem cell transplantation.
ConclusionTaken together, precise histopathologic diagnostics including the analysis of surface markers are fundamental for the optimal treatment of NLPHL. Outcomes with stage- and risk-adapted approaches are excellent. Most patients experience long-term survival.