TABLE 3A International Prognostic Index Prognostic factors

Performance status >ECOG1 LDH > normal Extranodal sites >1 Stage 3 or 4

Note: The table shows the identified prognostic variables.

TABLE 3B CR with Initial Therapy and the Chance of OS at Five Years

Risk group Number of risk factors CR% OS%

Low-intermediate 2 67 51

High-intermediate 3 55 43

High 4-5 44 26

Abbreviations: CR, complete response rate; OS, overall survival.

TABLE 4A Follicular Lymphoma International Prognostic Indexa Prognostic factors

Age >60

Ann Arbor Stage 3-4 Number of nodal sites >4 LDH > normal Hemoglobin <12

aThe table shows the identified risk factors.

TABLE 4B Five-Year and 10-Year Survival Probabilities According to the Number of Risk Factors

Risk group

Number of risk factors

5 yr OS (SE) (%)

10 yr OS (SE) (%)

Low

0-1

90.6 (1.2)

70.7 (2.7)

Intermediate

2

77.6 (1.6)

50.9 (2.7)

High

>3

52.5 (2.3)

35.5 (2.8)

Abbreviation: OS, overall survival; SE, standard error.

Abbreviation: OS, overall survival; SE, standard error.

of hemorrhagic and thrombotic manifestations are the goals of therapy. It is also important to limit therapy-related complications. In some situations, no therapy is indicated, as long as followup with a hematologist occurs. In PRV, eventually there may be progression to the postpolycythemic spent phase, a time during which increasing transfusion requirements develop. Additional complications that may develop include myeloid metaplasia with MF or AML (67). In appropriate circumstances, younger patients may be considered for a potentially curative allogeneic stem-cell transplant.

CML initial therapy is now imatinib mesylate (Gleevec) (64). The median duration of response has not yet been established and initial hematologic complete response rates are extremely high. Cytogenetic and molecular response rates are also high, but complete molecular responses are very rare. The only established curative therapy is an allogeneic transplant, but morbidity and mortality concerns remain. Currently, a trial of imatinib with consideration of an allogeneic transplant in appropriate candidates with a suboptimal initial response is indicated for most patients. Imatinib appears to alter the natural history of CML. Prior to imatinib, the median survival was four years, with typical progression from chronic phase to accelerated phase and ultimately, blast crisis.

CLL is a disorder with a widely variable prognosis. Many patients follow an asymptomatic course and may never require treatment. Others can be identified as higher risk based on the clinical presentation, course, or molecular features of the leukemic cells.

With conventional chemotherapy, the disorder is incurable, but an allogeneic transplant can be curative in selected patients.

AML and ALL can also be stratified in terms of risk status at the time of disease presentation. Cytogenetic abnormalities are particularly important in this regard. A risk-adapted approach to transplant is followed for eligible patients.

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