0521779407-01 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:45
Acute Lymphoblastic Leukemia 35■Patients with poor-risk ALL associated with t(9;22) have benefited
from incorporation of imatinib mesylate (Gleevec) into their regi-
men. Allogeneic stem cell transplantation should be vigorously pur-
sued for patients with this specific diagnosis, and additional second-
generation ABL kinase inhibitors are under development.
■Patients with mature B-cell ALL (L3) associated with charac-
teristic chromosomal abnormalities, including t:(8;14)(q24;q32);
t(2;8)(p12;q24); or t(8;22)(q24;q11), often rapidly progress unless
therapy is started expeditiously; patients with this type of ALL are
susceptible to massive tumor lysis, necessitating adequate metabolic
preparation and intervention during therapy.
■Patients with mature B-cell ALL (L3) are treated with intensive
chemotherapy (including cyclophosphamide, high-dose methotrex-
ate, and cytosine arabinoside in addition to vincristine, ifosfamide,
dexamethasone, VM-26 and Adriamycin) combined in an alternat-
ing series of courses administered over 6 months. This has markedly
improved the outcome in this disease. CNS treatment is also manda-
tory.
■Treatment of T-cell ALL has markedly improved with the addition
of cyclophosphamide and cytosine arabinoside during induction
therapy; current CR rates are ranging from 82% to 93%, with LFS
at 3 years now 50%, making this a favorable subtype of ALL.
■Stem Cell Transplantation
■In patients with failure to achieve response by weeks 4 to 6 or those
with adverse cytogenetics (e.g., t:9;22 and t:4;11), the plan should
include a vigorous search for allogeneic stem cell transplantation
(either HLA-matched sibling or a matched unrelated donor [MUD])
during first remission; strategies must include participation in ongo-
ing clinical trials.
■In fact, all patients with ALL should be encouraged to participate in
ongoing clinical trials based upon their risk assessment for initial
response and duration.Management Summary
■Patients with ALL should be assigned to specific treatment protocols
based upon an assessment of their risk for response to therapy and
duration of response.
■Therapeutic strategies can be divided into phases of required treat-
ment, including induction, consolidation, intensification, main-
tenance therapies; essentially all patients need to be treated for
CNS disease; some patients can be treated with disease-specific