Adults with acute lymphocytic leukaemia (ALL) generally get long-term chemotherapy (chemo). Doctors have begun to utilise more intense chemo regimens in recent years, which has resulted in increased responses to therapy. However, these regimens are more likely to result in adverse effects such as low white blood cell counts. Other medications may be required to help prevent or treat these adverse effects.
Treatment is usually divided into three stages:
- Incorporation (remission induction)
- Reorganization (intensification)
The overall treatment time is generally around two years, with the maintenance phase taking up the majority of that time. Depending on the subtype of ALL and other prognostic variables, treatment may be more or less intensive.ALL has the potential to spread to the brain and spinal cord. This has sometimes already occurred by the time ALL is identified. This spread is discovered through a lumbar puncture (spinal tap), when leukaemia cells are discovered in the cerebrospinal fluid (CSF), a liquid that surrounds the brain and spinal cord. This is addressed in more detail below.
Even if leukaemia cells are not identified in the CSF at the time of diagnosis, they may spread there later. As a result, central nervous system (CNS) prophylaxis — therapy that reduces the danger of leukaemia spreading to the region around the brain or spinal cord – is an important element of ALL treatment. This is also covered in greater depth further down.
The purpose of induction chemotherapy is to put the leukaemia into remission (complete remission). This implies that leukaemia cells are no longer detected in bone marrow samples (through a bone marrow biopsy), normal marrow cells are restored, and blood counts return to normal levels. However, remission does not always imply cure, as leukaemia cells may still be present 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:
- Dexamethasone or prednisone
- An anthracycline drug such as doxorubicin (Adriamycin) or daunorubicin
Some regimens may include cyclophosphamide, L-asparaginase (or pegaspargase), and/or high doses of methotrexate or cytarabine (ara-C) as part of the induction phase, depending on the patient’s prognostic variables. This first month of therapy is intense and necessitates numerous medical visits. Because significant infections or other problems might develop, you may spend some or all of this period in the hospital. It is critical to take all medications exactly as directed. Complications can be life-threatening at times, but with recent advancements in supportive care (nursing care, nourishment, antibiotics, growth factors, red blood cell and platelet transfusions when needed, and so on), they are far less prevalent than in the past. In most cases, induction chemotherapy induces remission in leukaemia. However, because leukaemia cells may still be present in the body, further therapy is required.CNS therapy or prophylaxis: Treatment is required to prevent leukaemia cells from spreading to the CNS (CNS prophylaxis) or to treat the leukaemia if it has already spread to the CNS (CNS treatment). This is frequently begun during induction and continues through the rest of the therapy stages. It might contain one or more of the following:
Chemotherapy is administered directly into the CSF (called intrathecal chemotherapy). Methotrexate is the most often utilized medication, however, cytarabine or a steroid such as prednisone may also be used. Intrathecal chemotherapy can be administered via a lumbar puncture (spinal tap) or via an Ommaya reservoir (as discussed in the surgery section). High-dose IV methotrexate, cytarabine, or other chemo drugs
If the leukemia goes into remission, the following step usually consists of another very brief course of chemo with many of the same medicines used during induction treatment. This usually lasts a few months. Typically, the medicines are administered in large dosages to ensure that the therapy remains quite intensive. Typically, CNS prophylaxis/treatment is continued at this period. Patients whose leukemia cells carry the Philadelphia chromosome are also given a targeted medication such imatinib. Some remission patients, such as those with specific ALL subtypes or other poor prognostic characteristics, are nonetheless at significant risk of the leukemia returning (coming back). Instead of conventional chemo, doctors may recommend an allogeneic stem cell transplant (SCT) at this time, especially if you have a sibling or sister who is a suitable donor match. An autologous SCT may also be a possibility. The dangers and advantages of a stem cell transplant must be carefully assessed for each patient depending on their individual circumstances, as it is not apparent if they are beneficial for all patients. Patients seeking this treatment should consider going to a clinic that has performed a large number of stem cell transplants.
Following consolidation, the patient is usually started on a methotrexate and 6-mercaptopurine maintenance chemotherapy regimen (6-MP). This may be taken with other medications like as vincristine and prednisone in some situations.A targeted medication like imatinib is frequently included in the treatment of ALL patients whose leukaemia cells carry the Philadelphia chromosome.
Maintenance typically lasts around two years. Typically, CNS prophylaxis/treatment is continued at this period.
What if the leukaemia does not respond to therapy or recurs?
If the leukaemia is resistant, meaning it does not respond to the first therapy (which happens in approximately 10% to 20% of patients), newer or more intense chemo treatments may be attempted, albeit they are less likely to succeed. Patients with B-cell ALL may benefit from monoclonal antibodies such as blinatumomab (Blincyto) or inotuzumab ozogamicin (Besponsa).If the leukaemia can be placed into at least partial remission, a stem cell transplant may be attempted. Clinical studies of novel therapeutic strategies may also be explored.
If leukaemia goes into remission after initial therapy but subsequently returns (relapses or recurs), it is more likely to do so in the bone marrow and blood. On rare occasions, the brain or spinal fluid will be the first site where it recurs. In some situations, additional chemotherapy (chemo) may be used to bring the leukaemia back into remission, however this remission is unlikely to continue. The treatment strategy may be determined by how quickly the leukaemia returns after the initial therapy. If a relapse occurs after a lengthy period of time, the same or a comparable treatment may be used to attempt a second remission. Immunotherapy may also be a possibility for certain people. For example, for certain individuals with B-cell ALL, a monoclonal antibody such as blinatumomab (Blincyto) or inotuzumab ozogamicin (Besponsa) may be a possibility, whereas CAR T-cell treatment may be an option for patients aged 25 and younger. ALL Philadelphia chromosome patients who were on a targeted therapy like imatinib (Gleevec) are frequently shifted to a new targeted drug.The chemotherapy medication nelarabine (Arranon) may be beneficial for people with T-cell ALL. If a second remission is feasible, most doctors would recommend a stem cell transplant if possible. I f the leukaemia does not go away or returns, further chemo is unlikely to be beneficial in the long run. If a stem cell transplant is not a possibility, a patient may wish to think about participating in a clinical trial of newer therapies.
It may become obvious at some time that further therapy, even in clinical trials, is highly unlikely to cure leukaemia. At that point, the focus of therapy may change to keeping the leukaemia and its symptoms under control for as long as feasible rather than attempting to cure it. This is known as palliative care or supportive care. Pain may occur as the disease spreads to the bone marrow. It is critical that you are as relaxed as possible. Radiation and suitable pain-relieving medications are two treatments that may be beneficial. If aspirin and ibuprofen do not relieve the pain, harsher opioid medications such as morphine are likely to be effective. Low blood counts and tiredness are other frequent signs of leukaemia.To help address these issues, medications or blood transfusions may be required. Medicines and high-calorie food supplements can be used to alleviate nausea and lack of appetite. Antibiotics can be used to treat any infections that arise.