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Acute lymphoblastic leukemia in children
Last reviewed: 23.04.2024
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Acute lymphoblastic leukemia is a group of clinically heterogeneous clonal malignant neoplasms from lymphocyte precursor cells, usually having certain genetic and immunophenotypic characteristics. Secondary anomalies of cell differentiation and / or proliferation lead to increased production and accumulation of lymphoblasts in the bone marrow and infiltration of lymph nodes and parenchymal organs. In the absence of treatment of acute lymphoblastic leukemia quickly becomes a fatal disease.
[Epidemiology
More than 80% of all leukemias in children are of lymphoid origin, of which 80% are tumors from B lymphocyte progenitors, 1% are tumors from mature B cells. About 15% come from T-lymphocytes, less than 5% have an indeterminate cellular origin.
Acute lymphoblastic leukemia is the most common oncological disease of childhood, accounting for about 25% of all malignant neoplasms in pediatrics. The incidence in developed countries is 30-40 cases per 1,000,000 children.
Symptoms of acute lymphoblastic leukemia
The main clinical signs of acute lymphoblastic leukemia are weakness, fever, malaise, pain in the bones and / or joints, hemorrhagic syndrome (bleeding of the mucous membranes of the oral cavity, cutaneous hemorrhages), pallor. Fever is usually associated with a bacterial, viral, fungal or protozoal (less common) infection, especially in children with severe neutropenia (less than 500 neutrophils per μL). Weakness occurs as a result of anemia and intoxication.
Recurrence of acute lymphoblastic leukemia
A victorious point in the treatment of acute lymphoblastic leukemia in children can be put only after a significant improvement in the results of treatment of relapses. Compared with the results of treatment of primary patients, survival of children with relapses of acute lymphocytic leukemia remains low, 5-year survival of these patients does not exceed 35-40%. The chances of recovery directly depend on the development of new approaches in polychemotherapy, options for bone marrow transplantation, etc. Separate and combined, bone marrow and extramedullary (with the defeat of the central nervous system, testicular, with infiltration of other organs), very early (within 6 months from the establishment diagnosis), early (up to 18 months after diagnosis) and later (18 months after diagnosis) relapses.
Diagnosis of acute lymphoblastic leukemia
Diagnosis of acute lymphoblastic leukemia is based on the history, physical examination and laboratory studies.
Laboratory diagnostics
General blood count: the number of white blood cells can be normal, reduced or elevated; often, though not always, they reveal blast cells; hyporegenerative normochromic anemia and thrombocytopenia are characteristic.
Biochemical blood test: characterized by an increase in LDH activity; also determine the indicators of kidney and liver function.
Myelogram: bone marrow puncture should be performed at a minimum of two points (in children under 2 years old it is the heel bone or tuberosity of the tibia, in older children - the posterior and anterior awns of the iliac bones) for the collection of a sufficient amount of diagnostic material. It is desirable to take a material intake under general anesthesia. It is necessary to make 8-10 smears from each point, and also collect material for immunophenotyping, cytogenetic and molecular-genetic studies.
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Treatment of acute lymphoblastic leukemia
The basic principles of treatment of acute lymphoblastic leukemia in children have been developed in the US back in the late 1960s. In fact, they have not changed to date. Modern treatment of acute lymphoblastic leukemia consists of several main phases: induction of remission with the use of three or more agents administered within 4-6 weeks, multi-agent consolidation ("fixing") of remission and maintenance therapy, usually using antimetabolites for 2- 3 years. A mandatory component is the prevention and treatment of neiroleukemia. Given the poor penetration of drugs through the blood-brain barrier, as early as 1965, it was proposed to use specific therapy aimed at sanation of the central nervous system.
Prognosis for acute lymphoblastic leukemia
Each of the modern protocols for the treatment of acute lymphoblastic leukemia poses its problems, the solution of which is poured into the general international course to optimize the therapy of this disease. For example, in the Italian version of the BFM-AIEOP protocol, the researchers left cranial irradiation only for children with hyperleukocytosis exceeding 100,000 cells per μL and with the T-cell variant of acute lymphocytic leukemia, while achieving adequate control over the occurrence of neurorecidivitis.