Nomenclature and classifications

Modern use of immunologic markers often detects neoplastic lymphoid cells in the blood of patients presenting with a mass of solid lymphoma. If such cells are present in numbers sufficient to give an abnormally high lymphocyte count, the case can be regarded as one of leukemia. Conversely, a patient presenting with a frankly leukemic picture might be troubled later in the disease by solid accumulations of neoplastic cells. Clearly there is no sharp dividing line between solid lymphomas and lymphoid leuk-emias, as has been recognized in the recent Revised European American Lymphoma (REAL) classification. However, in most current texts, and in this entry, the two forms are treated separately in deference to current clinical practice.

Another problem of nomenclature arises with neoplasms of Ig-secreting B lymphoid cells, of which the most important representatives in humans are multiple myeloma and macroglobulinemia. These have some features sufficiently distinctive to lead to their being considered apart from lymphomas in most texts. In this entry the term lymphoma will be used to embrace all neoplasms of lymphoid cells, including the leukemias and Ig-secreting tumors.

To classifying lymphomas there is no end. The varying roles of viruses, the often multiple genomic lesions, and the interactions with normal tissues combine to present tumors of bewildering variety, with apparently unique features associated with each patient. Nevertheless those common features which can be discerned have led to insights into pathogenesis, and often reflect significant differences in prognosis and responses to therapy, so that the efforts of lymphoma taxonomists have been well justified. Each person approaching the subject must decide upon the depth of classification which their interests dictate. The basic classification of the commoner human lymphomas set out in Table 1 provides the major headings in this entry. Much further along the spectrum of complexity are the 50 or so types and subtypes of lymphoma listed in the well-researched and eminently expandable REAL classification. Unfortunately a vast amount of clinical data are locked into the Working Formulation, a classification of non-Hodgkin lymphomas (NHI.) in widespread use since 1982 which omits references to cell lineages and has proved poorly adaptable to recent advances.

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