A systematic literature review of the clinical and epidemiological burden of acute lymphoblastic leukaemia (ALL)

Our goal was to identify and summarize the published literature pertaining to the incidence, prevalence, mortality, aetiology, clinical diagnosis, and management of acute lymphoblastic leukaemia (ALL). Acute lymphoblastic leukaemia represents 12% of all leukaemia cases, with a worldwide incidence projected to be 1-4.75 per 100,000 people. Italy, the United States (US), Switzerland, and Costa Rica are the countries with the highest incidence of ALL. Hereditary link, genetic defects, and possibly radiation or chemical exposures are listed amongst the most significant risk factors. Acute lymphoblastic leukaemia is predominantly a disease of childhood, but it affects adults as well. It accounts for 80% of all leukaemia cases in children. The incidence is slightly higher in men than in women and greater in white people than in black people. In 2003 in the US, there were an estimated 5800 deaths from ALL. Presenting signs and symptoms of ALL are fairly non-specific and include fever, anaemia, petechiae, and bone and joint pain. Staging of the disease and patient risk profile are routinely performed to define ALL subtypes and guide management. Chemotherapy, cranial radiation in patients with high-risk disease, and stem cell transplantation for selected patients are the prevalent therapies. Complete remission rates are high, especially amongst children (even 100%); however, long-term survival at 10 years (event-free survival) is in the range of 63% for children and 25-35% for adults. This implies that there is still a strong need for new therapies to maintain remission and prolong survival. Future treatment strategies may be driven by the patient's minimal residual disease status, a measure that more precisely defines remission, prognosis, responsiveness to therapy, and expected long-term survival.