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17 - Precursor lymphoid neoplasms

from Section 2 - Neoplastic hematopathology

Published online by Cambridge University Press:  03 May 2011

By
Mihaela Onciu
Edited by
Maria A. Proytcheva
Mihaela Onciu
Affiliation:
St. Jude Children's Research Hospital
Maria A. Proytcheva
Affiliation:
Northwestern University Medical School, Illinois
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Summary

Definition

B- and T-lymphoblastic leukemias/lymphomas comprise a family of malignant lymphoid neoplasms that morphologically and immunophenotypically recapitulate the features of early lymphoid precursors of B- or T-lineage, respectively [1]. By convention, neoplasms with predominant extramedullary involvement (defined as lacking bone marrow involvement or replacing less than 25% of the marrow cellularity) are classified as lymphoblastic lymphomas (LBLs). The remaining cases, showing bone marrow involvement of 25% or more, are staged and treated as acute lymphoblastic leukemias (ALL).

Epidemiology

Acute leukemias represent the most common type of pediatric cancer (31% of all childhood malignancies). Approximately 85% of all pediatric acute leukemias are ALLs [2]. In children, ALL shows a peak of incidence between the ages of two and five years [3]. The annual standardized rate for ALL per million population for children aged 0 to 14 years varies by geographic region, ranging from 16–18 in India and China, to 38–41 in the United States and Canada, to 46 in Costa Rica [2]. There is a slight male predominance (male : female ratio 1.2–1.5) and a striking excess incidence among white children (with the standardized rate for pediatric ALL in the United States being 20 for black children and 38 for white children) [3]. Acute lymphoblastic leukemia occurs with increased frequency in patients with certain genetic syndromes, including Down syndrome, Bloom syndrome, neurofibromatosis type 1, and ataxia telangiectasia [4].

Type
Chapter
Information
Diagnostic Pediatric Hematopathology , pp. 323 - 344
Publisher: Cambridge University Press
Print publication year: 2011

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To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

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