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Acute lymphoblastic leukaemia (ALL) is a cancer of the blood and bone marrow. Bone marrow is spongy tissue that fills the cavity of the long bones consisting of fat, red blood cells, and white blood cells. ALL affects the immature white blood cells.
Acute leukaemia accounts for up to 30% of all childhood malignancies.
ALL is the most common form of cancer in children. Repeated infection, bleeding, or fatigue/pallor not responding to treatment should raise the suspicion of ALL[1].
This type of cancer is classified by one of two subtypes.[2][3][4][5]
B-cell- produce antibodies to fight infections.
Common ALL (50% of cases)
Early Pre-B ALL (10% of cases)
Pre-B ALL (10% of cases)
Mature B-cell ALL (4% of cases)
T-cell- assist B-cells in producing antibodies to fight infections.
Age: Younger patients have a better prognosis compared to older patients. The cure rate for children is approximately 80% and only 40% for adults.
Initial white blood cell (WBC) count: Patients diagnosed with a WBC count below 50,000 tend to do better than patients with higher WBC counts.
ALL subtype: The subtype of T cell or B cell affects prognosis. Patients with T-cell ALL tend to have a better prognosis than those with mature B-cell ALL.
Response to chemotherapy: Patients who achieve complete remission within 4 to 5 weeks of starting treatment tend to have a better prognosis. Patients who do not achieve remission at any time have a poor prognosis. Evidence of the minimal presence of leukaemia cells in the bone marrow may also positively affect prognosis.
Etiology
There is solid evidence that ALL has a genetic component.
This is evidenced by many distinct translocations associated with the disease and a higher prevalence of the disease in monozygotic twins.
ALL incidence has also been found to be higher in patients with immunodeficiency disorders such as Down syndrome, neurofibromatosis type 1, Bloom syndrome, and ataxia telangiectasia[1]
Epidemiology
Photo courtesy of MedimanageThe estimated number of ALL new cases in the United States is approximately 6000 per year.
ALL is primarily a disease of children younger than 6 years with a slight male predominance.
About 85% of ALL cases are B-cell in origin, and 15% are T-cell in origin.
T-cell ALL is more common in males, African Americans and adolescents.
T-ALL accounts for approximately 25% of adults ALL[1].
Characteristics/Clinical Presentation
Most signs and symptoms of ALL mimic those of the flu. However, with all these signs and symptoms will not improve. Signs and symptoms include[2][4][5]:
Drugs for high-risk patients: Daunorubicin, Cytarabine
Other drugs that may be given early: Methotrexate, 6-mercaptopurine.
Diagnostic Tests/Lab Tests/Lab Values
Diagnostic tests and results for ALL include[2][5]:
Blood test: These tests reveal an increased number of white blood cells, a decreased number of red blood cells (anaemia) and platelets (thrombocytopenia). A blood test may also reveal blast cells. Blast cells are immature blood cells in the bone marrow.
Red blood cells: These cells carry oxygen throughout the body. A low number of red blood cells can lead to feeling tired or weak, being short of breath and looking pale (anaemia).
White blood cells: These cells fight infections. A low number of white blood cells can lead to fever and frequent infections that are hard to treat.
Platelets: Platelets control bleeding. A low number of platelets can lead to slow healing, easy bruising or bleeding and tiny red spots under the skin (petechiae).
Leukaemia cells: An increased number of these cells can cause pain in the bones or joints, lack of appetite, headache or vomiting
Bone marrow test: A needle is used to remove a sample of bone marrow from the hipbone to look for cancerous cells. Through this process, doctors can determine if the cells originated from the B lymphocytes or T lymphocytes according to certain changes in the cancer cells.
Lumbar puncture or spinal tap: A sample of spinal fluid is collected to determine if the cancerous cells have spread.
Imaging: X-ray and computerized tomography (CT) scans can help determine if the cancer has spread to other parts of the body.
Medical Management
Treatment for ALL can span from 2 ½ to 3 ½ years depending on each situation. General treatment for ALL is broken down into the following 4 phases[2][4][5][9]:
Induction therapy: The purpose of this phase is to achieve remission by killing most of the cancer cells in the blood and bone marrow. During the first month, the patient may require many doctor's visits due to the increased risk of infections. Patients often receive 3 drugs for the first month of treatment which include:
Chemotherapy drugs injected intrathecally
Steroids
A fourth drug in the anthracycline class is typically added for high-risk patients.
Consolidation therapy/Post-remission therapy: During this more intensive phase, the goal is to destroy any remaining leukaemia cells in the central nervous system. This phase of chemotherapy lasts 4 to 8 weeks typically. Intrathecal therapy is continued during this phase.
Maintenance therapy: This low-dose treatment is given to prevent cancer cell re-growth. During the first few months of this phase, most treatments include 1 to 2 treatments similar to the initial induction. These 4-week intense treatments are called re-induction.
Preventive treatment to the spinal cord: Some cancer cells in the central nervous system can not be destroyed by chemotherapy drugs given by oral or intravenous means. During this phase, chemotherapy drugs are injected directly into the spinal cord fluid.
During these 4 phases the specific types of treatments include:
Chemotherapy: Chemotherapy is normally used in the induction therapy stage to kill the cancer cells. This drug can also be used in the consolidation and maintenance phases. All patients need spinal taps to inject chemotherapy into the cerebrospinal fluid (CSF) to kill any leukaemia cells that may have spread to the brain and spinal cord. This intrathecal chemotherapy is usually given twice during the first month and four to six times during the next one to two months. It is repeated less often during the rest of the consolidation and maintenance. A possible side effect of intrathecal chemotherapy is epileptic seizures during treatment.
Catheter placement involving a tube placed in a large vein for patients that require many IV treatments
Intrathecal injections into the cerebral spinal fluid or through a catheter placed under the scalp
Targeted drug therapy: These drugs attack specific abnormalities that cause the cancer cell growth.
Radiation therapy: This treatment uses high-powered beams to destroy cancer cells. This is typically used when the cancer has spread to the central nervous system. Along with intrathecal chemotherapy, high-risk patients and those with leukaemia cells detected in their CSF may be given radiation therapy to the brain and spinal cord. Doctors try to avoid this treatment if possible, especially in younger children, because it may cause problems in growth and development.
Stem cell transplant: This transplant may be used for patients at risk or currently going through a relapse. This procedure replaces cancer bone marrow through chemotherapy or radiation with healthy bone marrow from a compatible donor allowing re-establishment of healthy stem cells.
Physical Therapy Management
Therapy is determined on an individual basis depending on clinical presentation and response to medical intervention.[7]
↑ 7.07.17.27.37.4Goodman, C., Boissonault, W. Pathology: Implications for the Physical Therapist. Saunders. Philadelphia, Pennsylvania. Third edition. 2009.
↑Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for referral. Saunders, St. Louis, Missouri. Fourth Edition. 2007.
