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Lymphoproliferative disorders: CT findings in immunocompromised children
L F Donnelly1, D P Frush, K W Marshall
1Department of Radiology, Duke University Medical Center, Durham, NC 27710, USA.
Insights
Epstein-Barr virus-induced lymphoproliferative disorders in children show varied CT imaging and distribution. Mortality is linked more to the type of immune dysfunction than disease spread, especially in transplant recipients.
Area of Science:
- Pediatric Radiology
- Immunology
- Oncology
Background:
- Epstein-Barr virus (EBV) can cause lymphoproliferative disorders (LPDs) in immunocompromised children.
- Understanding the imaging features and prognostic factors of EBV-LPDs is crucial for timely diagnosis and management.
Purpose of the Study:
- To evaluate the CT imaging characteristics, disease distribution, and outcomes of pediatric EBV-LPDs.
- To correlate imaging findings and disease patterns with the type of immunocompromised state and patient prognosis.
Main Methods:
- Retrospective review of medical records and CT imaging studies from four tertiary children's medical centers.
- Analysis of pathologically proven EBV-LPD cases in patients under 20 years old.
- Statistical analysis (Fisher's exact test) to identify trends between CT appearance, disease distribution, immune status, and prognosis.
Main Results:
- Twenty-seven pediatric cases of EBV-LPD were identified, with a mean age of 7 years 8 months.
- Common CT findings included lymphadenopathy, focal masses, and organ enlargement; abdominal involvement was most frequent.
- Mortality rates (44%) were significantly associated with the type of immune dysfunction (e.g., 100% in bone marrow transplant recipients) rather than disease extent.
Conclusions:
- Pediatric EBV-LPDs exhibit diverse CT appearances, distribution patterns, and outcomes.
- In solid organ transplant recipients, EBV-LPDs often localized to the anatomical region of the transplant.
- Prognosis is more strongly influenced by the underlying immune deficiency than the spatial distribution of the LPD.
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.