Advances in biology, diagnostics, and treatment of Hodgkin's disease.
Hodgkin and Reed-Sternberg cells are derived from germinal center B cells. Amplification of identical rearranged and mutated immunoglobulin genes from different Hodgkin and Reed-Sternberg cells from the same patient also answered the question of the malignant nature because the clonality-the key criterion of malignancy-of Hodgkin and Reed-Sternberg cells was hereby shown. In addition, it could be demonstrated that Hodgkin and Reed-Sternberg cells do not only expand clonally within 1 affected lymph node, but also clonally disseminate in advanced-stage disease and relapse even after clinical complete remission. Recent publications demonstrate that a probably small subset of Hodgkin disease exists with T-cell derivation of the respective Hodgkin and Reed-Sternberg cells. The management of Hodgkin's disease is undergoing a paradigm shift as a result of very effective drug regimens that are capable of inducing high remission rates, the use of combined chemoradiotherapy with involved-field irradiation in early stages, the introduction of effective salvage chemotherapy of relapsed Hodgkin's disease with peripheral stem cell transplantation, a better understanding of prognostic factors, economic constraints, and a more sensitive realization of the magnitude of late treatment mortality.
Küppers
Ralf
R
Institute for Cell Biology, Tumor Research, University of Duisburg-Essen, Germany.
Yahalom
Joachim
J
Josting
Andreas
A
D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D015322:Gene Rearrangement, B-Lymphocyte / Q000235:genetics / Q000276:immunology; D018858:Germinal Center / Q000276:immunology / Q000473:pathology; D006689:Hodgkin Disease / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000628:therapy; D006801:Humans; D009154:Mutation / Q000276:immunology; D011379:Prognosis; D016539:Reed-Sternberg Cells / Q000276:immunology / Q000473:pathology; D012074:Remission Induction; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology