Objective: The objective was to evaluate the CT imaging appearance, distribution of disease, type of immunocompromised state, and outcome of children with Epstein-Barr virus-induced lymphoproliferative disorders.
Materials and methods: Medical records and imaging studies (from four tertiary children's medical centers) were reviewed for pathologically proven cases of lymphoproliferative disorders in patients less than 20 years old. Trends between the CT imaging appearance, distribution, and type of immunocompromised state and prognosis were noted and analyzed with Fisher's exact test.
Results: Twenty-seven cases were identified (mean age, 7 years 8 months). Eighteen children had undergone solid organ transplantation (heart, n = 9; liver, n = 7; kidney, n = 2), and four had undergone bone marrow transplantation. Five patients had primary immunodeficiencies. The CT appearance of lymphoproliferative disorders varied and included lymphadenopathy, focal mass or masses, and diffuse infiltration and enlargement of organs without focal mass. The distribution of disease included abdomen (n = 17), chest (n = 10), neck (n = 8), and brain (n = 1). In eight of nine heart transplant recipients, the disease predominantly involved the chest and neck, whereas in all seven liver transplant recipients, the disease was isolated to the abdomen (p = .001). The overall mortality rate of 44% was less related to anatomic extent (multiorgan, 46%; localized, 43%) than to type of immune dysfunction (p = .001): bone marrow transplantation (100%), primary immunodeficiency (80%), heart transplantation (55%), liver transplantation (0%), and kidney transplantation (0%).
Conclusion: Lymphoproliferative disorders in children had a variable distribution, imaging appearance, and outcome. However, in recipients of solid organ transplants, the disease tended to occur in the anatomic region of the transplant. Mortality rates were more closely related to the type of underlying immune dysfunction than to distribution of disease.