Lymphoproliferative Disorders 

  • Author: Stuart S Winter, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Nov 24, 2009
 

Background

Lymphoproliferative disorders (LPDs) in children represent a heterogeneous group of expanding, monoclonal or oligoclonal, lymphoid cells that occur in the setting of immune dysfunction. The risk of true malignancy in affected children is significantly higher than the risk in immunocompetent children. Treatment must be tailored to the child's underlying immune disorder, to the aggressiveness of the clone, and to the likelihood of causing clinically significant toxicity.

In this article, underlying immunodeficiency disorders are reviewed in the context of the type of lymphoproliferative disorder encountered.

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Pathophysiology

See Causes.

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Epidemiology

Frequency

United States

Lymphoproliferative disorders occur in children with immunodysfunction. Because this is a heterogeneous disease group, the incidence rate is difficult to estimate.

Mortality/Morbidity

Mortality and morbidity in children vary considerably and depend on the underlying immunodeficiency syndrome.

Race

Overall, no significant racial predilection has been reported.

Sex

The overall male-to-female ratio is 1:1, except for X-linked immunodeficiency syndromes, which primarily affect male individuals; however, X-linked immunodeficiency syndrome occasionally affects female individuals. In scenarios such as this, mutations in the gene that encodes NFkappaB essential modifier (NEMO), which can be inherited in autosomal dominant fashion, lead to immunodeficiency syndromes in members of both sexes.

Age

Lymphoproliferative disorders can occur in any age group but are relatively uncommon in infants and toddlers. They become progressively more common with age.

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Contributor Information and Disclosures
Author

Stuart S Winter, MD  Associate Professor, Department of Pediatrics, University of New Mexico Health Sciences Center

Stuart S Winter, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, New Mexico Pediatric Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, University of Cincinnati College of Medicine; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, Cincinnati Children's Hospital Medical Center

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

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