Acute lymphoblastic leukemia natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Carlos A Lopez, M.D. [3]
Overview
Prognosis has improved from a 0% to 20-75% survival rate largely due to the continuous development of clinical trials and improvements in bone marrow transplantation (BMT) and stem cell transplantation (SCT) technology. The prognosis for acute Lymphoblastic leukemia differs between individuals depending on a wide variety of factors such as gender, ethnicity, age, blood cell count, dissemination and genetic involvement.
Natural History, Complications, and Prognosis
Natural history
If left untreated, of patients with Acute lymphoblastic leukemia may progress to develop infection, bleeding, infertility, and metastasis to other organs.
Prognosis
The overall cure rate in children is 85%, and about 50% of adults have long-term disease-free survival.[1][2]
Advancements in medical technology and research over the past four decades in the treatment of acute lymphocytic leukemia has improved the overall prognosis significantly from a 0% to 20-75% survival rate, largely due to the continuous development of clinical trials and improvements in bone marrow transplantation (BMT) and stem cell transplantation (SCT) technology.
It is worth noting that medical advances in recent years, both through matching the best treatment to the genetic characteristics of the blast cells and through the availability of new drugs, are not fully reflected in statistics that usually refer to five-year survival rates.
The prognosis for acute Lymphoblastic leukemia differs between individuals depending on a wide variety of factors:
Gender
- Females tend to fare better than males.
Ethnicity
- Caucasians are more likely to develop acute leukemia than African-Americans, Asians and Hispanics and tend to have a better prognosis than non-Caucasians.
Age
- Age is a significant factor in children with acute lymphoblastic leukemia and may be an important prognostic factor in adult with acute lympoblastic leukemia as well. In one study, overall the prognosis was better in patients younger than 25 years; another study found a better prognosis in patients younger than 35 years. These findings may in part, be related to the increased incidence of the Ph1 in older acute lymphoblatic leukemia patients a subgroup associated with poor prognosis. Children between 1-10 years of age are most likely to be cured.
Blood cell count
- White blood cell count at diagnosis of less than 50,000/µl.
Dissemination
- Whether the cancer has spread to the brain or spinal cord
Genetic involvement
- Down's Syndrome tend to have a genetic involvement.
- Chromosomal abnormalities: Chromosomal abnormalities including aneuploidy and translocations, have been described and may correlate with prognosis. In particular, patients with Ph1-positive t(9;22) acute lymphoblastic leukemia have a poor prognosis and represent more than 30% of adult cases. Bcr-abl-rearranged leukemias that do not demonstrate the classical Ph1 carry a poor prognosis that is similar to those that are Ph1-positive. Patients with Ph1-positive acute lymphoblastic leukemias are rarely cured with chemotherapy, although long-term survival is now being routinely reported when such patients are treated with combinations of chemotherapy and Bcr-abl tyrosine kinase inhibitors. Two other chromosomal abnormalities with poor prognosis are t(4;11), which is characterized by rearrangements of the myeloid lymphoid leukemia gene and may be rearranged despite normal cytogenetics, and t(9;22). In addition to t(4;11) and t(9;22), compared with patients with a normal karyotype, patients with deletion of chromosome 7 or trisomy 8 have been reported to have a lower probability of survival at 5 years.In a multivariate analysis, karyotype was the most important predictor of disease-free survival.
Cytogenetic subtypes with worse prognosis
- A translocation between chromosomes 9 and 22, known as the Philadelphia chromosome, occurs in about 20% of adult and 5% in pediatric cases of acute lymphoblastic luekemia.
- A translocation between chromosomes 4 and 11 occurs in about 4% of cases and is most common in infants under 12 months.
- Not all translocations of chromosomes carry a poorer prognosis. Some translocations are relatively favorable. For example, hyperdiploidy (>50 chromosomes) is a good prognostic factor.
Central nervous system involvement
- As in childhood acute lymphoblastic leukemia, adult patients with acute lymphoblastic luekemia are at risk of developing central nervous system involvement during the course of their disease. This is particularly true for patients with L3 (Burkitt) morphology. Both treatment and prognosis are influenced by this complication.
Celular morphology
- Patients with L3 morphology showed improved outcomes, as evidenced in a completed cancer and Leukemia Group B study, when treated according to specific treatment algorithms. This study found that L3 leukemia can be cured with aggressive, rapidly cycling lymphoma-like chemotherapy regimens.
5 Year survival
- Between 2004 and 2010, the 5-year relative survival of patients with acute lymphoblastic leukemias was 70%.[3]
- When stratified by age, the 5-year relative survival of patients with acute lymphoblastic leukemias was 71.3% and 12.2% for patients <65 and ≥ 65 years of age respectively.[3]
References
- ↑ Harrison's Principles of Internal Medicine, 16th Edition, Chapter 97. Malignancies of Lymphoid Cells. Clinical Features, Treatment, and Prognosis of Specific Lymphoid Malignancies.
- ↑ "National Cancer Institute".
- ↑ 3.0 3.1 Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.