These are more common in children than adults (~6000 total cases/year). The majority of cases have tumor cells that appear to be of thymic origin, and pts may have mediastinal masses. Pts usually present with recent onset of signs of marrow failure (pallor, fatigue, bleeding, fever, infection). Hepatosplenomegaly and adenopathy are common. Males may have testicular enlargement reflecting leukemic involvement. Meningeal involvement may be present at diagnosis or develop later. Elevated LDH, hyponatremia, and hypokalemia may be present, in addition to anemia, thrombocytopenia, and high peripheral blood blast counts. The leukemic cells are more often FAB type L2 in adults than in children, where L1 predominates. Leukemia diagnosis requires at least 20% lymphoblasts in the marrow. Prognosis is adversely affected by high presenting white count, age >35 years, and the presence of t(9;22), t(1;19), and t(4;11) translocations. HOX11 expression identifies a more favorable subset of T cell acute lymphoblastic leukemia.
Treatment: Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma Successful treatment requires intensive induction phase, CNS prophylaxis, and maintenance chemotherapy that extend for about 2 years. Vincristine, l-asparaginase, cytarabine, daunorubicin, and prednisone are particularly effective agents. Intrathecal or high-dose systemic methotrexate is effective CNS prophylaxis. Long-term survival of 60-65% of pts may be achieved. The role and timing of bone marrow transplantation in primary therapy are debated, but up to 30% of relapsed pts may be cured with salvage transplantation. |
Section 6. Hematology and Oncology