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Rudolph's PediatricsRudolph's Pediatrics

Section 24. Neoplastic Disorders > 

Part 2. Hematologic Malignancies > 

Chapter 449. Acute Lymphoblastic Leukemia

Acute Lymphoblastic Leukemia: Introduction

James B. Nachman
Topics Discussed: acute lymphocytic leukemia; b-cell development; leukemia; leukemia, b-cell, acute; leukemia, lymphocytic, acute, childhood; leukemia, t-cell; medical oncology; t-cell development.
Sections: References.
Excerpt:"Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, accounting for almost 25% of cancer diagnoses seen in children younger than age 15 years. Patients with more than 25% lymphoid blasts in the bone marrow are considered to have leukemia regardless of the presence of extramedullary disease. Patients who have an extramedullary lymphoid mass and 5% to 25% lymphoid blasts in the marrow are considered to have lymphoblastic lymphoma with marrow involvement. There are approximately 2400 new cases of ALL diagnosed in children and adolescents less than 20 years of age each year in the United States.1The peak incidence of ALL is in the 2- to 6-year-old age group. The incidence of ALL appears to be highest in Hispanic children and lowest in African American children.2In the developmental pathway of lymphocytes (see Fig. 449-1), maturation to the mature B-cell and T-cell state occurs without the necessity of antigenic stimulation—so-called antigen independent differentiation. When a B or T cell combines with its specific antigen, proliferation and differentiation occur, resulting in long-lived memory cells for antigen recognition and plasma cells which secrete antibody or T cells with various effector functions, such as cytotoxicity, B-cell help, cytokine secretion, and so on. In B-lineage acute lymphoblastic leukemia (ALL), approximately 98% of patients show a B-precursor immunophenotype. The rare patient with mature B-cell leukemia has a..."
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