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140 The development of cancers like chronic lymphocytic leukemia and aggressive non-Hodgkin’s lymphoma following an autoimmune muscle disease and its treatment is well described in the medical literature; see Ola Landgren et al., “Acquired immune-related and inflammatory conditions and subsequent chronic lymphocytic leukaemia,” British Journal of Haematology 139 (2007), pp. 791–798; A. Créange et al., “Inflammatory neuromuscular disorders associated with chronic lymphoid leukemia: Evidence for clonal B cells within muscle and nerve,” Journal of the Neurological Sciences 137 (1996), p. 35–41; Takao Endo et al., “Polymyositis-dermatomyositis and non-Hodgkin’s lymphoma,” Internal Medicine 32 (1993), pp. 487–489; László Váróczy et al., “Malignant lymphoma-associated autoimmune disease: A descriptive epidemiological study,” Rheumatology International 22 (2002), pp. 233–237; Whon-Ho Chow et al., “Cancer risk following polymyositis and dermatomyositis: A nationwide cohort study in Denmark,” Cancer Causes and Control 6 (1995), pp. 9–13; R. J. Evans, H. H. B. Hilton, “Polymyositis associated with acute monocytic leukemia: Case report and review of the literature,” CMAJ 91 (1964), pp. 1272–1275; Catherine L. Hill et al., “Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study,” Lancet 357 (2001), pp. 96–100.

144 Although chronic lymphocytic leukemia is a very common blood disorder, there is still debate among experts about when it is optimal to initiate treatment and what that treatment should be; key factors in these decisions include the rate of rise in the white count and the presence or absence of prognostic molecular markers like ZAP-70, CD38, and unmutated immunoglobulin Bh genes. In general, blood counts are performed at three-month intervals along with a physical examination. Many hematologists track the rate of increase in blood lymphocyte count as well as any new symptoms like weight loss, fever, or bleeding. Clinical studies show that survival of patients with a blood lymphocyte doubling time shorter than twelve months is significantly less than those with longer doubling times. Some clinicians decide to begin treatment in this setting, although others do not treat based on the doubling time alone. In addition, the absolute level of blood lymphocyte count needs to be considered, since doubling of the count from 10,000 to 20,000 in less than twelve months in a patient who is at an early stage of disease and free of symptoms does not have the same significance as a doubling of the count from 75,000 to 150,000; see Nicholas Chorazzi, Kanti R. Rai, M. Ferrarini, “Mechanisms of disease: Chronic lymphocytic leukemia,” NEJM 352 (2005), pp. 804–815; Thorsten Zenz et al., “From pathogenesis to treatment of chronic lymphocytic leukaemia,” Nature Reviews Cancer 10 (2010), pp. 37–50; John G. Gribben, “How I treat CLL up front,” Blood 115 (2010), pp. 187–197; Stefano Molica, Antonio Alberti, “Prognostic value of lymphocyte doubling time in chronic lymphocytic leukemia,” Cancer 60 (1987), pp. 2712–2716; Emili Monserrat et al., “Lymphocyte doubling time in chronic lymphocytic leukaemia: Analysis of its prognostic significance,” British Journal of Haematology 62 (1986), pp. 567–575; Michael Hallek et al., “Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: A report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute—Working Group 1996 guidelines,” Blood 111 (2008), pp. 5446–5456.

145 Paul Peterson’s chronic lymphocytic leukemia became resistant to standard treatment regimens. He faced the option of receiving more chemotherapy that might control the leukemia for a relatively short duration of time (usually months) or undergoing bone marrow transplantation. The clinical issues involved in making the choices are reviewed in Emili Montserrat et al., “How I treat refractory CLL,” Blood 107 (2006), pp. 1276–1283.

147 The field of decision analysis was established in the 1950s. A now classical publication by