Foon K A, Gale R P
Division of Clinical Immunology, Roswell Park Memorial Institute, Buffalo, NY 14263.
Nouv Rev Fr Hematol (1978). 1988;30(5-6):385-8.
Chronic lymphocytic leukemia (CLL) is usually stable over months to years however a small proportion of cases may transform to more aggressive forms. These transformations include diffuse lymphoma (Richter syndrome), prolymphocytic transformation, acute lymphoblastic leukemia and multiple myeloma. There are a variety of techniques to determine whether the transformation represents a clonal evolution of the original CLL or an independent disease. These include cytogenetic analysis, immunoglobulin gene rearrangement by Southern blot analysis, and anti-idiotypic antibodies. While identity can be determined by gene rearrangement and anti-idiotypic antibodies, lack of identity can occur because of somatic mutation within a single clone. Thus, even with optimal techniques it is difficult to definitively exclude lack of identity in some instances. In most cases where transformation has taken place, definitive studies were not performed. In instances where detailed data are available, most do not suggest clonal evolution. Further studies are needed to allow definite conclusions.
慢性淋巴细胞白血病（CLL）通常在数月至数年期间保持稳定，然而一小部分病例可能会转变为更具侵袭性的形式。这些转变包括弥漫性淋巴瘤（里氏综合征）、幼淋巴细胞转化、急性淋巴细胞白血病和多发性骨髓瘤。有多种技术可用于确定这种转变是代表原始CLL的克隆进化还是一种独立疾病。这些技术包括细胞遗传学分析、通过Southern印迹分析进行免疫球蛋白基因重排以及抗独特型抗体。虽然可以通过基因重排和抗独特型抗体确定一致性，但由于单个克隆内的体细胞突变，也可能出现不一致的情况。因此，即使采用最佳技术，在某些情况下也很难明确排除不一致的情况。在大多数发生转变的病例中，并未进行确定性研究。在可获得详细数据的情况下，大多数数据并不提示克隆进化。需要进一步研究才能得出明确结论。
