New developments in extracutaneous lymphomas.
The recently proposed World Health Organization classification of neoplastic diseases of the lymphoid tissues is based on the principles of the Revised European-American Classification of Lymphoid Neoplasms introduced in 1994. Use of these classifications implies a new approach to lymphoma diagnosis, especially because of the inclusion of clinical data among which the site of involvement (nodal v extranodal) is very important. Recent technical advances allowing molecular biological investigations on the single cell level helped gain new insights into the cellular origin of B-cell lymphomas. Tumor cells of the majority of B-cell non-Hodgkin's lymphomas (NHL) harbor somatically mutated immunoglobulin variable region genes, and are therefore derived from germinal center B cells or their descendants. The same is true for Hodgkin's disease, which (at least in the majority of cases) is a germinal center derived B-cell lymphoma. Significant news on the molecular pathogenesis of NHL include the prognostically relevant dichotomy of B-CLL, the involvement of translocations affecting 3q27 in 20% to 40% of diffuse large B-cell lymphomas (DLBCL), the prognostical implication of the t(2;5) in anaplastic large cell lymphoma, and detection of the t(11;18) in gastric mucosa-associated lymphoid tissue (MALT)-type lymphoma. A major step forward with regard to gastric MALT-type lymphoma therapy was the discovery of a causal relationship between Helicobacter pylori infection and lymphomagenesis. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy remains the golden standard for DLBCL treatment.
Chott
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Department of Clinical Pathology, University of Vienna, Austria.
Raderer
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D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001402:B-Lymphocytes / Q000378:metabolism* / Q000648:ultrastructure; D002471:Cell Transformation, Neoplastic; D006689:Hodgkin Disease / Q000235:genetics; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics; D016393:Lymphoma, B-Cell / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics; D009154:Mutation; D011379:Prognosis; D000069283:Rituximab; D013274:Stomach Neoplasms / Q000235:genetics; D014178:Translocation, Genetic
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab