Chronic Lymphocytic Leukemia (CLL) - Rai Classification

8/13/01

Question: What is the Rai classification for staging CLL?

[NOTE: the original publication is in #4]

<1>

Unique Identifier: 94223921 / PubMed Identifier: 8170175

Authors: Call TG. Phyliky RL. Noel P. Habermann TM. Beard CM. O'Fallon WM. Kurland LT.

Institution: Division of Hematology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905.

Abstract: OBJECTIVE: To determine whether the stage at the time of diagnosis of chronic lymphocytic leukemia (CLL) had changed during a 55-year period. DESIGN: We conducted a study of the cohort of residents of Olmsted County, Minnesota, who had been diagnosed as having CLL during the period from 1935 through 1989. MATERIAL AND METHODS: By analysis of medical records, patients with CLL were characterized by Rai stage, absolute lymphocyte count, age at diagnosis, need for therapy, and reported cause of death in nonsurvivors. Trends for these variables were analyzed by decade throughout the study period. RESULTS: The overall annual incidence rate of CLL per 100,000 population in Olmsted County increased from 2.6 in the 1935 through 1944 period to 5.4 in the 1975 through 1984 period; however, the increasing rate was found only for those 50 years of age or older and was especially dramatic for those 75 years old or older. Analysis of Rai stage over time demonstrated an increase in the proportion of cases diagnosed as Rai stage 0. In addition, the median absolute lymphocyte count decreased, the median time to initiation of therapy increased, and the median age of patients with Rai stage 0 CLL at the time of diagnosis increased over time. Overall, 54% of patients had received therapy for CLL by the time of last follow-up. Among the nonsurvivors, CLL was documented as the underlying or a contributing cause of death in 69%. CONCLUSION: The overall increase in CLL was thought to be due to enhanced methods of early diagnosis and improved health care for the elderly population. Thus, artifact may best explain the observed trend, although we cannot exclude the possibility of an actual increase in incidence rates over time.

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Unique Identifier: 87129383 / PubMed Identifier: 3814821

Authors: Lee JS. Dixon DO. Kantarjian HM. Keating MJ. Talpaz M.

Abstract: Three hundred twenty-five previously untreated patients with chronic lymphocytic leukemia were analyzed to identify significant prognostic factors for survival. Univariate analysis identified the following characteristics associated with survival: (1) clinical characteristics: age, race, sex, performance status, lymphadenopathy, and hepatosplenomegaly; (2) hematologic parameters: WBC count, absolute lymphocyte and granulocyte counts, hemoglobin level, and platelet count; and (3) biochemical parameters: serum albumin, calcium, uric acid, lactate dehydrogenase, alkaline phosphatase, BUN, and creatinine. Multivariate regression analysis in a randomly selected training subset of 217 patients demonstrated that the combination of uric acid, alkaline phosphatase, lactate dehydrogenase, external lymphadenopathy, and age had the strongest predictive relation to survival time. The resulting model was validated in the remaining independent subset of 108 patients and led to classification of patients into low, intermediate, and high-risk groups with five-year survival rates of 75%, 59%, and 14%, respectively, and with distinctively different annual mortality rates (P less than .01). Both the regression model and Rai staging were highly effective in identifying risk groups among the entire patient population (P less than 0.001). Overall the regression model was superior to Rai staging in defining prognostic risk groups. In addition, it was able to separate patients into significantly different risk categories within each Rai stage, thus improving on the prognostic prediction of individual patients with chronic lymphocytic leukemia.

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Unique Identifier: 83050596 / PubMed Identifier: 7139586

Authors: Skinnider LF. Tan L. Schmidt J. Armitage G.

Abstract: Review of 745 cases of chronic lymphocytic leukemia show that clinical staging by either the Rai or Binet method is very valuable in assessment of patients at the time of diagnosis. There is a marked decrease in survival with advancing stage (19.9 years median survival for Rai Stage 0, and 2.5 years and 2.7 years for Rai Stages 3 and 4, respectively). The definition of anemia as Hb less than 11 g/dl as in the Rai staging method appears to give slightly better discrimination among the stages than the Binet staging procedure. Patients with splenomegaly alone (Binet stage 2), however, form a small but distinct group that should be recognized. These is little to be gained by subdividing further according to size of the lymph nodes.

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Unique Identifier: 75184472 / PubMed Identifier: 1139039

Authors: Rai KR. Sawitsky A. Cronkite EP. Chanana AD. Levy RN. Pasternack BS.

Abstract: A method of clinical staging of chronic lymphocytic leukemia (CLL) has been proposed which is based on the concept that CLL is a disease of progressive accumulation of nonfunctioning lymphocytes: stage O, bone marrow and blood lymphocytosis only; stage 1, lymphocytosis with enlarged nodes; stage II, lymphocytosis with enlarged spleen or liver or both; stage III, lymphocytosis with anemia; and stage IV:lymphocytosis with thrombocytopenia. Analysis of 125 patients. in the present series showed the following median survival times (in months) from diagnosis: stage 0, is greater than 150; stage I 101; stage II, 71; stage III, 19; stage IV, 19, The median survival for the entire series was 71 mo. The prognostic significance of the stage remained even after adjustment was made for age and sex. However, both sex and age were shown to be poor predictors of survival after adjustment for stage. The method of staging proved to be a reliable predictor of survival whether used at diagnosis or during the course of the disease. The proposed staging system was an equally accurate indicator for survival when applied to two other previously published studies of large series of patients