New Insights into Malignant B-Cell Disorders

Mature B-cell disorders comprise numerous and very different entities including all B-cell lymphomas (NHL) subgroups , chronic lymphocytic leukemia (CLL), and plasma cell dyscrasias [1]. Growing knowledge on disease biology has led to the development of new drugs, while innovative diagnostic techniques have resulted in improvement of diagnosis, establishment of new prognostic factors, and the recognition of new entities. Original research and review articles published in this special issue highlight some interesting aspects of this topic. Herein, we will briefly present these papers and the scientific background they rely on. The treatment of symptomatic multiple myeloma (MM), a severe plasma cell dyscrasia, with considerable morbidity and a reported, until recently, disappointing median survival of 3–5 years, has incredibly changed over the last decade, for the benefit of patients. Prolonged remission duration and improved survival with a better quality of life can be now observed, while the notion of an eventual cure does not seem so improbable anymore [2, 3]. The new drugs that led to the aforementioned improvements were not " chemother-apeutic " agents in the classical sense of the term because they were all " biology-modulating " factors. The first three " so-called " new drugs that constitute today the backbone of MM treatment are (i) bortezomib, the first-in-class reversible proteasome inhibitor that downregulates the three major disease expansion mechanisms, namely, cell escape from apoptosis, neoangiogenesis, and osteoclast activation and (ii) thalidomide and (iii) thalidomide-analog lenalidomide that both commonly exert antiangiogenic, anti-inflammatory, and immunomodulatory functions. Today, next generation novel agents are already available and others under evaluation, while treatment with monoclonal antibodies (mAbs) against disease specific targets has been developed and appears promising [4]. mAb treatment alone or in combination with chemother-apy (immunochemotherapy) begun much earlier for the treatment of B-NHL and CLL with the introduction in the late 90s of a humanized monoclonal antibody against CD20, rituximab, resulting in a significant improvement of patients' response rates. Afterward, intense research efforts were made and are still ongoing, to exploit the better knowledge of disease pathogenesis and to develop biologically active drugs in the field of B-NHL and CLL. With regard to CLL, which is a relatively common leukemia of the elderly in western countries, it behaves indolently in more than half of patients but may have more aggressive behavior that should be immediately and effectively treated. Conventional therapy included alkylators, purine analogs, and corticosteroids, while the introduction of rit-uximab …