Rai (2) notes that the roots of the Rai staging system for CLL can be traced back to the work of Galton presented in a Burroughs Wellcome Lecture in 1965 and subsequently published by the Canadian Medical Association in 1966 (27). Galton presented data on 88 CLL patients followed for 15 yr classifying them into four groups:
i. Neither lymph node nor spleen enlargement ii. Only lymph node enlargement iii. Only spleen enlargement iv. Both lymph node and spleen enlargement.
The distributions of these four groups (i-iv) were 19, 27, 8, and 56, respectively. Galton further noted that patients with high ALC tended to be group iv and that 71% of patients in group iv had evidence of bone marrow compromise. On the other hand, only 56% of patients with splenomegaly and 24% of patients with lymphadenopathy had symptoms related to their CLL. As already noted, Galton also described two patterns in the rise in ALC: type I (aggressive) and type II (indolent). Rai further notes that Wintrobe and colleagues (26) reported that all the physical and laboratory evidence of disease tended to be more severe in CLL patients surviving for less than 5 yr. However, he credits Dameshek (28) with proposing the first formal staging system in 1967: stage 1, asymptomatic blood and marrow lymphocytosis; stage 2, symptomatic generalized lymphadenopathy with variable splenomegaly; stage 3, symptomatic bulky disease with frequent infections; and stage 4, fevers, infections, pneumonias, and increasing anemia.
On the basis of these observations, in 1975 Rai et al. (48) proposed the following five stages:
Stage 0 blood and marrow lymphocytosis Stage I lymphocytosis with lymphadenopathy
Stage II lymphocytosis with splenomegaly and/or hepatomegaly with or without lymphadenopathy Stage III lymphocytosis with anemia (Hgb < 11 g/dL); nodes, spleen, or liver may or may not be enlarged
Several observations can be made from this progression of events, but first it should be noted that the Rai staging system has been proved to have prognostic value. When this system was tested on their own data and confirmed on the data of Boggs et al. (26) and Hansen et al. (29), the following median values were found: 150, 101, 71, 19, and 19 mo for Rai stages 0-IV, respec tively. It would appear that the Rai stage 0, Galton's group (i), and Dameshek's asymptomatic stage 1 are identical. Galton's group (ii), Dameshek's stage 2, and Rai stage I are likewise nearly identical. Isolated splenomegaly, Galton's group (iii), and Rai stage II are also identical. Galton's group (iv), and Dameshek's stage 3 are identical. With regard to Rai stages III and IV, Galton does not specify these two groups, and Dameshek's stage 4 would be equivalent to the Rai stage III. Thus the physical examination and clinical laboratory findings of CLL are recognized in the Rai Staging System. The lymphocytosis is systemic and progressive, involving the blood and bone marrow, and there is a progressive, generalized, symmetrical lymphadenopathy involving the spleen and the liver and resulting in anemia, thromboyctopenia, and death.
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