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Figure 23.2 Morphology and histology of CLL in peripheral blood and lymph node. (a) Peripheral blood smear of a patient with CLL (1000 X magnification). Typical CLL lymphocytes are small and well differentiated, with round nuclei and clumped chromatin. (b) Peripheral blood smear of a patient with PLL. Prolymphocytes comprise greater than 55% of cells and are larger with dispersed chromatin, prominent, single nucleoli, and abundant cytoplasm (1000 X magnification). (c) Peripheral blood smear of a patient with CLL, illustrating "smudge cells" (500 Xmagnification)

b a c the Rai staging system,25,26 used in the United States, and the Binet staging system,27 used more commonly in Europe (Table 23.3). The Rai staging system was initially proposed as a five-stage system, with stage 0 characterized by lymphocytosis, stage I by adenopathy, stage II by organomegaly, stage III by anemia, and stage IV by thrombocytopenia.25 This five-stage system was simplified into three stages, consisting of low risk (stage 0), intermediate risk (stages I and II), and high risk (stages III and IV).26 The estimated median survival for patients with low-risk disease was >10 years, for intermediate-risk disease 7 years, and for high-risk disease 1.5-4 years. The Binet system is a three-stage system and is based on the number of lymph node sites involved and the presence or absence of cytope-nias. Generally, patients progress through the stages with progression of their disease, in the absence of treatment.

The prognostic significance of stage is more obvious for patients with advanced-stage disease. However, the majority of patients are diagnosed with early stage disease, of whom about half will have an indolent clinical course and the other half will have a more progressive course. Therefore, significant effort has gone into identifying prognostic factors for patients with early stage disease that predict for rapidly progressive disease. Recently identified important prognostic factors that have been identified include immunoglobulin heavy-chain variable (IgVH) gene mutational status,28,29 expression of surface CD38,29,30 and ZAP-70 expression.31-34 These prognostic factors are most useful for counseling patients with early stage disease, as approximately half of these patients will be nonprogressors with an indolent clinical course and half will have progressive disease, requiring treatment in a short period.

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