Sebahoun G, Blanc A P, Sainty D, Gastaut J A, Carcassonne Y
Sem Hop. 1980;56(9-10):414-7.
Two cases of CLL terminating in Richter's syndrome and in blastic crisis raise the problem of the physiopathological signification of these unusual complications. Richter's syndrome is described as a "histiocytic" lymphoma or a Hodgkin's disease supervening on a CLL. It does not seem that these two pictures have actually to be distinguished since clinical and haematological aspects are not different, and histopathologic studies show always atypical aspects with difficulty of an accurate diagnosis. Cases of blastic crisis of CLL are very uncommon. They correspond to the transformation of a CLL in acute lymphoblastic leukaemia. These two terminal complications of CLL suggest they may be interpreted as two pictures of the transformation of the same initial B cellular clone. This hypothesis, highly probable for the Richter's syndrome, has been actually demonstrated for the acute transformation of CLL.
两例慢性淋巴细胞白血病(CLL)分别发展为里氏综合征(Richter's syndrome)和原始细胞危象,这引发了这些罕见并发症的生理病理学意义问题。里氏综合征被描述为叠加在慢性淋巴细胞白血病上的“组织细胞性”淋巴瘤或霍奇金病。由于临床和血液学方面并无差异,且组织病理学研究总是显示出非典型特征,难以进行准确诊断,所以这两种表现似乎实际上无需区分。慢性淋巴细胞白血病的原始细胞危象病例非常罕见。它们相当于慢性淋巴细胞白血病转变为急性淋巴细胞白血病。慢性淋巴细胞白血病的这两种终末并发症提示,它们可能被解释为同一初始B细胞克隆转化的两种表现。这一假设对于里氏综合征极有可能成立,实际上已在慢性淋巴细胞白血病的急性转化中得到证实。