[Hodgkin disease--pathogenesis and therapy]

Hodgkin's lymphoma exerts characteristics of a true hematopoietic neoplasia as well as of an atypic immune response. In affected lymphatic tissue a minority of malignant Hodgkin-Reed Sternberg [H-RS] cells is surrounded by a majority of reactive, nonmalignant cells. Clinical features and biological studies suggest a pronounced, but inefficient, T-cell response against a yet undefined [viralS/B] target antigen expressed on H-RS cells. The role of the Epstein-Barr virus [EBV] which can be detected in the H-RS cells in about 50% of cases remains to be defined. By micromanipulation of single H-RS cells from frozen sections and subsequent enzymatic amplification of nucleic acids [single cell PCR] for the first time a monoclonal B-cell origin of H-RS cells could be demonstrated in several cases of Hodgkin's disease. Therapeutic strategies in Hodgkin's disease, in contrast to non-Hodgkin's lymphoma, are not based on the histological subtype, but rather on precise determination of the stage of disease. Treatment of choice in limited disease stages without risk factors is radiation therapy. Intermediate stages are treated with combined chemo- and radiation therapy, advanced stages with intensive chemotherapy [subsequent radiation only on bulk tumors]. Thus, about 85% of all patients suffering from Hodgkin's disease, can be cured definitely. High-dose chemotherapy followed by autologous stem cell transplantation in relapsed Hodgkin's lymphoma is currently proofed in clinical studies.