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Overview of non-Hodgkin lymphoma in children and adolescents

Amanda M Termuhlen, MD
Thomas G Gross, MD, PhD
Section Editor
Julie R Park, MD
Deputy Editor
Alan G Rosmarin, MD


Non-Hodgkin lymphoma (NHL) consists of a diverse group of malignant neoplasms of the lymphoid tissues variously derived from B cell progenitors, T cell progenitors, mature B cells, or mature T cells. Unlike in adults where low-grade, clinically indolent NHL subtypes predominate, most pediatric NHL cases are of high grade and have an aggressive clinical behavior [1]. Whenever possible, children with NHL should be treated in a comprehensive pediatric oncology center by a multidisciplinary team experienced in the diagnosis and care of children with cancer.

This topic will provide an overview of NHL in children and adolescents, focusing on issues that are of interest to primary care providers. The pathobiology of NHL and its diagnosis and management in adults are presented separately, as is an overview of Hodgkin lymphoma in children and adolescents. (See "Classification of the hematopoietic neoplasms" and "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Overview of Hodgkin lymphoma in children and adolescents".)


NHL is the fifth most common diagnosis of pediatric cancer in children under the age of 15 years, and it accounts for approximately 7 percent of childhood cancers in the developed world [2]. In the United States, approximately 800 new cases of pediatric NHL are diagnosed annually with an incidence of 10 to 20 cases per million people per year [3-6]. This incidence appears to be increasing overall, largely thought to reflect a rise in NHL among adolescents. The median age at diagnosis is approximately 10 years, and the incidence increases with age [2]. Lymphomas are rare in infants (≤1 percent) and account for approximately 4, 14, 22, and 25 percent of neoplasms in children age 1 to 4, 5 to 9, 10 to 14, and 15 to 19 years, respectively. There is a male predominance, and whites are more commonly affected than African Americans.

The incidence and distribution of specific NHL subtypes differs by population (eg, age, race) and geographical region. In general, the most common subtypes of pediatric NHL are derived from B cell progenitors. In the United States and other developed countries, the most common subtypes are Burkitt lymphoma, diffuse large B cell lymphoma, lymphoblastic T cell or B cell lymphoma, and anaplastic large cell lymphoma [6]. Other subtypes (eg, follicular lymphoma, marginal zone lymphoma) are less common, accounting for approximately 7 percent of pediatric NHL.

Both congenital and acquired immunodeficiency syndromes are associated with an increased risk of NHL. Congenital immunodeficiencies associated with NHL include common variable immunodeficiency, Wiskott-Aldrich syndrome, ataxia telangiectasia, and X-linked lymphoproliferative syndrome. Congenital immunodeficiencies may impact treatment decisions. As an example, the use of diagnostic tests and therapy involving x-rays and ionizing radiation should be limited in children with ataxia telangiectasia to minimize the risk of somatic mutations and subsequent malignancy. Acquired immunodeficiencies include HIV and the use of immunosuppressive medications. Post-transplant lymphoproliferative disorders can follow solid organ and hematopoietic cell transplantation. (See 'Lymphoproliferative disease in the immune compromised patient' below and "Epidemiology, clinical manifestations, and diagnosis of post-transplant lymphoproliferative disorders" and "Epidemiology, clinical manifestations, and diagnosis of post-transplant lymphoproliferative disorders", section on 'Epidemiology' and "Ataxia-telangiectasia".)


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Literature review current through: Sep 2016. | This topic last updated: Jan 14, 2016.
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