Lymphoproliferative Disorders

Updated: Apr 18, 2019
  • Author: Donna A Wall, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Overview

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 majority of disorders are autosomal recessive and affect both sexes equally with a male-to-female ratio of 1:1. A small but distinct population suffer from X-linked immunodeficiency syndromes, which almost exclusively affect male individuals; but may affect females if mutations in the gene that encodes NFkappaB essential modifier (NEMO) are involved, which is inherited in autosomal dominant fashion.

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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