暂无摘要。

Waldenstrom\'s macroglobulinemia (WM) is a malignant disorder of lymphoplasmacytic cells that produce a monoclonal immunoglobulin M (IgM). Since the original description by Jan Waldenström of three patients with symptoms of hyperviscosity due to circulating monoclonal IgM, the definition of WM has been controversial. Standardized criteria for diagnosis have now been proposed, including the presence of any IgM monoclonal protein and marrow and/or nodal lymphoplasmacytic cells. Although previous response criteria have generally incorporated parameters for monoclonal protein reduction and/or improvement of marrow/nodal involvement, specific and uniform response criteria are needed to facilitate comparisons of response, remission duration, progression-free survival, and overall survival in clinical trials similar to those previously established for other diseases such as chronic lymphocytic leukemia, lymphoma, and myeloma. This is of particular importance as new agents are developed and evaluated. This presentation represents consensus recommendations for uniform response criteria for use in assessing responses to treatment for patients with WM, which were prepared in conjunction with the Second International Workshop held in Athens, Greece during September 2002.

The detection and quantification of monoclonal free light chains in urine (Bence Jones protein, BJP) are thorny issues for the laboratorian. Immunoelectrophoretic techniques (immunofixation) allow the characterization of the two pathognomonic features of light chains: monoclonality and absence of heavy chains. Immunochemical methods such as nephelometry and turbidimetry are widely used in clinical practice to exclude the presence of BJP. However, these methods are limited by several metabolic and analytical problems. The accuracy of quantitative immunochemical methods is hampered by the heterogeneous molecular forms (fragments and polymers) of BJP and by the lack of reference materials, and the precision of the methods in clinically relevant regions of the dynamic range is poorly defined. Immunoelectrophoretic methods, especially immunofixation, are recommended because of their ability to demonstrate monoclonality and the absence of heavy chains. Immunofixation is also considered the best method to document the disappearance of the monoclonal protein (complete remission). The physiology of immunoglobulins and the clinical relevance of BJP are illustrated in the two appendices to this paper.