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Acute lymphoblastic leukaemia 

Acute lymphoblastic leukaemia
Chapter:
Acute lymphoblastic leukaemia
Author(s):

H. Josef Vormoor

, Tobias F. Menne

, and Anthony V. Moorman

DOI:
10.1093/med/9780198746690.003.0523
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date: 25 February 2021

Acute lymphoblastic leukaemia (ALL) is a malignant proliferation of lymphoid blasts, most commonly of B-lineage origin. The clinical symptoms and signs are either a consequence of bone marrow failure (infections, bruising, petechiae, pallor, and tiredness) or a consequence of the uncontrolled proliferation of the blasts (lymphadenopathy, hepatosplenomegaly, and cranial nerve palsies). Its peak incidence is in young children but ALL occurs at all ages. More than 80% of all affected children are cured with modern chemotherapy, but unfortunately the outcome of adults is much worse despite some improvements led by the introduction of paediatric-inspired protocols and tyrosine kinase inhibitors in BCR-ABL1-positive ALL. Standard chemotherapy for ALL consists of several months of intensive multidrug induction, consolidation and intensification chemotherapy (including steroids, vincristine, asparaginase and anthracyclines), intrathecal methotrexate to target blasts in the central nervous system, and low-intensity maintenance therapy (with oral 6-mercaptopurine and methotrexate) for up to 3 years. Treatment is stratified according to the response and other prognostic biomarkers (including genetics). Allogeneic haematopoietic stem cell transplantation is used predominantly in the relapse setting for children but in frontline therapy for adult patients to consolidate chemotherapy. Novel targeted small molecules and, in particular, immunotherapy are promising to offer new treatment options for patients with high-risk or relapsed disease.

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