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After publication of the Rai staging system and the general realization that patients with anemia and thrombocytopenia defined high-risk CLL, the question arose as how to define the nonanemic, nonthrombocytopenic patients who constitute 75% of CLL patients. It was felt that the large variability in this group was not well explained. The French CLL group, under the leadership of Binet, proposed in 1977, revised in 1981, and presented in 1981 a new classification based on three prognostic groups: C, B, and A (49-51). Group C was defined as anemia (Hgb < 10 g/dL) and/or thrombocytopenia (platelets < 100,000 ^L); group B as no anemia, no thrombocytopenia, three or more involved areas (counting as one each of the following: cervical, axillary, inguinal lymph nodes, whether unilateral or bilateral, spleen, and liver); and group A as no anemia, no thrombocytopenia, and less than three involved areas. In the study group that they used, the distributions of patients were 15% in group C, 35% in group B, and 50% in group A. The median survivals were 2 yr in group C, 7 yr in group B, and in group A survival did not seem to differ at the time from that of the French population of the same age and sex as noted by Binet in 1988 (52).

After the emergence of the Rai system, Montserrat and Rozman (53), in an analysis of prognostic factors in CLL, noted in 1988 that the widely used Rai staging system had been confirmed in more than seven or eight studies. They classified their 269 patients according to both the Rai and Binet systems, and their figures are shown for the sake of comparison (Figs. 30 and 31). They note the problems that still exist regarding the limitations of these staging systems: too many clinical stages in the Rai system; [it should be noted that in 1987 Rai (54) also revised the original five groups down to three groups]; lack of an explanation for median survival of Rai stage II patients being the same as the whole population of CLL patients; failure to separate out the causes of anemia and thrombocytopenia on the basis of marrow failure or autoimmune destruction as separate prognostic groups; and failure to identify progressive and quiescent clinical forms in either Rai stage 0/I or Binet stage A. In 1988 Binet (52) made an extensive comparative analysis of the Binet and Rai systems: the low-risk stages A (0), A (I), A (II); the intermediate-risk stages B (I), B (II); and the high-risk stages C (III) and C (IV). As would be expected, Binet stage A contains more better outcome patients (66%) than Rai stage 0 (31%); both Rai stage I and II patients can be further subdivided in into Rai stage IA and IB and Rai stage IIA and IIB, with prognostic significance. Rai stage IA contains two-thirds of the patients, and Rai stage IB contains one-third of the patients, with 5-yr survivals of 78% and 52%, respectively. Rai stage IIA contains one-third of the patients, and Rai stage IIB contains two-thirds of the patients, with 5-yr survivals of 68% and 38%, respectively. See Figs. 32 and 33 for a comparison of the Rai and Binet systems, and see Fig. 34 for the overall survival of Rai stage IA and IB, Rai stage IIA and IIB, and Rai stage IIIA, IIIB, and IIIC. Of course the Binet system confirms the poor prognostic outcome of the anemic and thrombocytopenic patient (52).

Fig. 30. Actuarial survival curves of 269 patients classified according to the Rai et al. staging system. Median survivals are as follows: stage 0, 126 mo; stage I, 92 mo; stage II, 53 mo; stage III, 23 mo; stage IV, 20 mo. (From ref. 53, Montserrat and Rozman, 1988, Fig. 1. With permission from Harwood Academic Publishers, Inc.)

Fig. 30. Actuarial survival curves of 269 patients classified according to the Rai et al. staging system. Median survivals are as follows: stage 0, 126 mo; stage I, 92 mo; stage II, 53 mo; stage III, 23 mo; stage IV, 20 mo. (From ref. 53, Montserrat and Rozman, 1988, Fig. 1. With permission from Harwood Academic Publishers, Inc.)

YEARS

Fig. 31. Actuarial survival curves of 269 patients classified according to the Binet et al. staging system. Median survivals are as follows: stage A, 128 mo; stage B, 47 mo; stage C, 24 mo. (From ref. 53, Montserrat and Rozman, 1988, Fig. 2. With permission from Harwood Academic Publishers, Inc.)

Years

Years

Fig. 32. Stages A, B, and C-COP cases. Overall survival according to Rai's staging (second interim analysis). (From ref. 52, Binet, 1988, Fig. 6. With permission from Harwood Academic Publishers, Inc.)

Fig. 32. Stages A, B, and C-COP cases. Overall survival according to Rai's staging (second interim analysis). (From ref. 52, Binet, 1988, Fig. 6. With permission from Harwood Academic Publishers, Inc.)

Years

Years

Fig. 33. Overall survival according to the (A, B, C) staging (second interim analysis); group C includes only COP treated patients. (From ref. 52, Binet, 1988, Fig. 5. With permission from Harwood Academic Publishers, Inc.)

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