Prognosis

The overall median survival is approximately 4 years, although individual survival may range from 1 to over 30 years. Several useful prognostic schemes have been reported that allow high-risk patients to be identified. The Lille scoring system (Table 10-6), based on two adverse prognostic factors, namely hemoglobin below 10g/dL and white cell count below 4 or above 30 X 109/L, is able to separate patients into three groups with low-, intermediate-, and high-risk disease.27 The Sheffield scheme (Table 10-7), by combining hemoglobin level, age, and kary-otype, identifies patients with median survival times that vary from 180 months (good risk) to 16 months (poor risk).28 These studies, however, included very few young patients, a fact that could limit their value when used to identify cases suitable for high-dose procedures. This criticism has been partly addressed by Cervantes and colleagues,29 who reported the outcome of 116 patients aged 55 years or younger at presentation. Survival was substantially longer than that reported for CIMF overall, with a median survival exceeding 10.5 years. Two risk groups were identified based on the presence of constitutional symptoms (fever, sweats, weight loss), a hemoglobin value of 10g/dL or lower and a blast cell count of 1 percent or higher in the peripheral blood. The median survival of patients with no, or only one, bad prognostic factor approached 15 years, whereas the minority of patients with two or three adverse factors survived for a median of less than 3 years. The relatively benign course of CIMF presenting in infancy has also been stressed30 and further emphasizes the need for caution when selecting patients for transplantation (as discussed below). The main causes of death in CIMF are infection, heart failure, thrombo-hemorrhagic events, and leukemic transformation. The last, which occurs in approximately 20 percent of patients, is associated with increasing cytogenetic abnormalities and responds poorly to therapy.

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