Acute Lymphocytic Leukemia Health Article

Treatment team

The treatment team consists of a hematologist/oncologist who directs care, oncology nurses familiar with administering chemotherapy, and often social workers, who can address both insurance issues and psychological support. The patient's regular physician should be kept informed of all cancer-related care. Because treatment is so prolonged, most patients have long-term intravenous catheters placed by a surgeon.

In many hospitals, a Child Life specialist will participate in the care of children with ALL. They ensure that children with cancer are seen, first and foremost, as children, organizing play times, providing distraction during scary procedures and giving parents some much-needed respite.

Clinical staging, treatments, and prognosis

ALL does not have a formal staging system, but treatment is different in different phases of the disease. These phases are often divided into untreated ALL, ALL in remission, and recurrent ALL. Conventional treatment for ALL consists of chemotherapy for disease in the bone marrow and treatment aimed at preventing central nervous system disease.

ADULTS.

The first phase of treatment is remission induction. The chemotherapeutic drugs typically include prednisone, vincristine, cytarabine, cyclophosphamide and asparaginase. Most are given intravenously and a few are given orally. Depending on the disease, these drugs can achieve a complete remission in 60% to 90% of adults. The relapse rate is higher in adults than in children. A 50% 3-year survival has been noted in some research series, and very aggressive treatment with multiple drugs has produced up to a 70% survival rate.

Adverse effects of these drugs include:

• bone marrow suppression
• anemia, pallor, fatigue, shortness of breath, and angina in older patients
• bleeding, bruising
• increased risk of infection
• hair loss (alopecia)
• mouth sores
• nausea and vomiting
• menopausal symptoms
• lower sperm counts
• tumor lysis syndrome, in which the dead cancer cells can harm healthy organs

Treatment that is directed at preventing central nervous system spread is called prophylactic. Because of the blood brain barrier, a physical and chemical barrier that prevents toxins from reaching the brain and spinal cord, chemotherapeutic drugs do not easily reach the central nervous system. Thus, chemotherapeutic drugs are administered directly into spinal fluid, which circulates around the brain and spinal cord. This is called intrathecal chemotherapy. The drugs are given by spinal tap or through an Ommaya reservoir, which is surgically inserted under the scalp. This reservoir empties into the spinal fluid around the brain.

Some patients receive prophylactic radiation therapy to the brain, in addition to or instead of intrathecal chemotherapy.

CHILDREN.

The treatment of ALL in children represents one of the great success stories of modern oncology. In contrast to adults, most children with cancer enter into research protocols, strict treatment regimens with careful follow-up that are built on the most successful aspects of earlier treatments. Childhood ALL now has an 80% long-term survival rate, due in large part to the extensive and widely disseminated research on the disease. Within the United States, research on ALL was conducted for many years under the auspices of either the Children's Cancer Group or the Pediatric Oncology Group. In 1998, recognizing the benefits of cooperation and collaboration, these two groups joined forces with the National Wilms' Tumor Study Group and the Intergroup Rhabdomyosarcoma Study Group to form the Children's Oncology Group.

Remission induction chemotherapy for children includes vincristine, a steroid, and asparaginase. Children at higher risk of relapse are often given daunomycin as well. The adverse effects of these drugs include bone marrow suppression, risk of infection, nausea, vomiting, hair loss, and mouth sores. Although these drugs can reduce sperm counts, most survivors of childhood ALL grow up to have normal fertility. The drugs can be administered intravenously or as oral preparations. Oral prednisone has a particularly unpleasant taste that is hard to disguise and parents must be vigilant to ensure that their children are taking their proper doses.

Like adults, children also receive prophylaxis against central nervous system spread. They receive multiple doses of intrathecal chemotherapy, with the drugs delivered directly to the spinal fluid through a lumbar puncture or spinal tap. Cranial radiation as central nervous system prophylaxis for children is infrequently used. Though once standard, brain radiation produced a high incidence of cognitive and learning disabilities, especially among those younger than five years old. Cranial radiation is reserved for those children felt to be at high risk of central nervous system disease, including those older than ten at the time of diagnosis, those with initial white blood cell counts of more than 50, 000 per microliter, and those with T-cell leukemia. Some high-risk children who enter remission rapidly with induction chemotherapy receive intrathecal chemotherapy alone, without radiation therapy.