ASSOCIATION BETWEEN LIVER-TRANSPLANTATION FOR LANGERHANS CELL HISTIOCYTOSIS, REJECTION, AND DEVELOPMENT OF POSTTRANSPLANT LYMPHOPROLIFERATIVE DISEASE IN CHILDREN

Objective: Langerhans cell histiocytosis (LCH) is an unusual indicatio n for orthotopic liver transplantation in children. Data from limited case reports suggest that orthotopic liver transplantation for LCH is associated with excellent survival rates and a low incidence of diseas e recurrence. However, in our experience, children who hal transplanta tion for LCH appeared to experience a high incidence of refractory rej ection and posttransplant lymphoproliferative disease (PTLD). Study de sign: Data from 398 liver transplants performed in 298 children younge r than 15 years of age were reviewed to determine the presence of risk factors for PTLD in patients with LCH and other causes of liver failu re. Results: The incidence of PTLD was significantly higher in childre n who received transplants for LCH compared with all indications (p < 0.001) and specific indications that were associated with the developm ent of PTLD (p < 0.002). hmong patients in whom PTLD developed, there was no significant difference in the incidence of primary Epstein-Barr virus infections in patients who receive transplantation for LCH (4/4 , 100%) versus all other indications (12/14, 86%). Children who had tr ansplantation for LCH were older than those who had transplantation fo r other indications (LCH median age 3.1 years, other indications 1 yea r). The incidence of rejection, especially refractory rejection, was g reater in patients who had transplantation for LCH (100% and 50%, resp ectively) compared with those who had transplantation for other indica tions (70% and 10%, p < 0.02 for refractory rejection). Conclusions: P atients who had transplantation for liver disease related to LCH exper ienced a 67% long-term survival (median follow up 5.8 years, range 2.1 to 7.5 years). Recurrent LCH occurred in only 33% of patients and tva s easily managed. However, PTLD developed in two thirds of these patie nts, perhaps in part because of the high incidence of refractory rejec tion. This series therefore demonstrates an association between a prim ary disease process and the development of PTLD. Although the data ind icate that children with LCH-induced liver failure benefit from transp lantation, special care must be exercised in screening for and preempt ive treatment of PTLD.