Childhood Hodgkin and Non-Hodgkin Lymphomas
Brigid G. Leventhal MD1
Gregory J. Kato MD2
1 Director of Clinical Research, The Johns Hopkins Oncology Center, Baltimore, Maryland 21205
2 Senior Clinical Fellow, Department of Pediatrics, The Johns Hopkins University School of Medicine, and The Johns Hopkins Oncology Center, 600 North Wolfe St, Baltimore, Maryland 21205
Lymphomas were recognized originally by Virchow as swellings of lymph nodes that were unrelated to tuberculosis or to other recognized pathology in the drainage area of the lymph node group. Lymphomas were not recognized initially as malignancies; proof of this came only in this century. Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), which together account for approximately 12% of childhood cancer, have become among the most curable of pediatric malignancies. Today, the projected cure rates for children in certain categories are as high as 90%. Recent advances in molecular biology have permitted immense strides in understanding the pathogenesis and biology of these two diseases.
FACTORS PREDISPOSING TO LYMPHOMAS
Of individuals with immune deficiency or defects of DNA repair function, 1% to 35% develop lymphomas. NHLs are highly associated with Wiskott-Aldrich syndrome, common variable immunodeficiency, severe combined immune deficiency syndrome, X-linked lymphoproliferative syndrome, and Bloom syndrome, and they are found in patients receiving immunosuppressive therapy following organ or bone marrow transplant. Both HLs and NHLs are seen with increased incidence in patients with ataxia-telangiectasia and acquired immunodeficiency syndrome.
Epstein-Barr virus, first strongly implicated epidemiologically in African Burkitt lymphoma, appears to have a role in the pathogenesis of at least 20% of B-cell lymphomas.
