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Castleman disease in a pediatric liver transplant recipient: a case report and literature review.

Bonatti HJ, Axt J, Hunter EB, Lott SL, Frangoul H, Gillis L, Correa H, Kelly B
Pediatr Transplant. 2012 16 (6): E229-34

PMID: 22032720 · DOI:10.1111/j.1399-3046.2011.01570.x

Castleman disease is a rare hematologic disorder, closely linked to the HHV-8, and most commonly observed in immunocompromised individuals. Thirteen months following a liver transplant for CPS-1 defect, a 15-month-old boy presented with fevers, anemia, and growth retardation. Abdominal CT scan showed splenomegaly and generalized lymphadenopathy. Histology of chest wall lymph nodes revealed a mixed CD3+ T-cell and CD20+ B-cell population with atretic germinal centers consistent with multicentric Castleman disease. Qualitative DNA PCR detected HHV-8 in the resected lymph node and in the blood, supporting the diagnosis. Immunosuppression was tapered, and he was transitioned from tacrolimus to sirolimus. His graft function remained stable, and repeat imaging showed regression of the lymphadenopathy. The child is living one yr after Castleman disease diagnosis with a well-functioning graft. Castleman disease is a potential complication of solid organ transplant and HHV-8 infection. Reduction in immunosuppression and switch to sirolimus may be an effective strategy to treat this condition.

© 2011 John Wiley & Sons A/S.

MeSH Terms (22)

Adolescent Adult Antigens, CD20 B-Lymphocytes Castleman Disease CD3 Complex Graft Survival Herpesvirus 8, Human Humans Immunosuppressive Agents Infant, Newborn Liver Failure Liver Transplantation Lymphatic Diseases Male Middle Aged Polymerase Chain Reaction Sirolimus Splenomegaly T-Lymphocytes Tacrolimus Tomography, X-Ray Computed

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