In this segment, Bijal D. Shah, MD, discusses risk stratification in patients with acute lymphoblastic leukemia (ALL). Raoul Tibes, MD, PhD, discusses the incorporation of asparaginase into adult regimens.

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Typical Treatment of Acute Lymphocytic Leukemia (ALL)
(Note: This information is about treating acute lymphocytic leukemia (ALL) in adults. To learn about ALL in children, see Leukemia in Children.)

The main treatment for acute lymphocytic leukemia (ALL) in adults is typically long-term chemotherapy (chemo). In recent years, doctors have begun to use more intensive chemo regimens, which has led to more responses to treatment. But these regimens are also more likely to cause side effects, such as low white blood cell counts. Patients may need to take other drugs to help prevent or treat these side effects.

Treatment typically takes place in 3 phases:

Induction (remission induction)
Consolidation (intensification)
Maintenance
The total treatment usually takes about 2 years, with the maintenance phase taking up most of this time. Treatment may be more or less intense, depending on the subtype of ALL and other prognostic factors.

ALL can spread to the area around the brain and spinal cord. Sometimes this has already occurred by the time ALL is first diagnosed. This spread is found when the doctor does a lumbar puncture (spinal tap) and leukemia cells are found in the cerebrospinal fluid (CSF), the liquid that surrounds the brain and spinal cord. The treatment of this is discussed below.

Even if leukemia cells aren't found in the CSF at diagnosis, it's possible that they might spread there later on. This is why an important part of treatment for ALL is central nervous system (CNS) prophylaxis – treatment that lowers the risk of the leukemia spreading to the area around the brain or spinal cord. This is also described in more detail below.

Induction
The goal of induction chemo is to get the leukemia into remission (complete remission). This means that leukemia cells are no longer found in bone marrow samples (on a bone marrow biopsy), the normal marrow cells return, and the blood counts return to normal levels. But a remission is not necessarily a cure, as leukemia cells may still be hiding somewhere in the body.

Induction chemo usually lasts for a month or so. Different combinations of chemo drugs might be used, but they typically include:

Vincristine
Dexamethasone or prednisone
An anthracycline drug such as doxorubicin (Adriamycin) or daunorubicin
Based on the patient’s prognostic factors, some regimens may also include cyclophosphamide, L-asparaginase (or pegaspargase), and/or high doses of methotrexate or cytarabine (ara-C) as part of the induction phase.

For ALL patients whose leukemia cells have the Philadelphia chromosome, a targeted drug such as imatinib (Gleevec) or dasatinib (Sprycel) is often included as well.

For patients who are older (typically over 65) or who have other serious health conditions, many of the same drugs are used for induction, although the doses of the drugs might need to be reduced.

This first month of treatment is intensive and requires frequent visits to the doctor. You may spend some or much of this time in the hospital, because serious infections or other complications can occur. It's very important to take all medicines as prescribed. Sometimes complications can be serious enough to be life-threatening, but with recent advances in supportive care (nursing care, nutrition, antibiotics, growth factors, red blood cell and platelet transfusions as needed, etc.), these are much less common than in the past.

Most often, leukemia goes into remission with induction chemotherapy. But because leukemia cells may still be hiding somewhere in the body, further treatment is needed.

CNS treatment or prophylaxis: Treatment needs to be given either to keep the leukemia cells from spreading to the CNS (CNS prophylaxis), or to treat the leukemia if it has already spread to the CNS. This is often started during induction and continued through the other phases of treatment. It may include one or more of the following:

Chemo injected directly into the CSF (called intrathecal chemotherapy). The drug used most often is methotrexate, but sometimes cytarabine or a steroid such as prednisone may be used as well. Intrathecal chemo can be given during a lumbar puncture (spinal tap) or through an Ommaya reservoir (as discussed in the surgery section).
High-dose IV methotrexate, cytarabine, or other chemo drugs
Radiation therapy to the brain and spinal cord
Consolidation (intensification)
If the leukemia goes into remission, the next phase often consists of another fairly short course of chemo, using many of the same drugs that were used for induction therapy. This typically lasts for a few months. Usually the drugs are given in high doses so that the treatment is still fairly intense. CNS prophylaxis/treatment is typically continued at this time.

A targeted drug like imatinib is also continued for patients whose leukemia cells have the Philadelphia chromosome.

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