Transplantation for congenital heart disease is associated with an increased risk of Epstein-Barr virus-related post-transplant lymphoproliferative disorder in children.

BACKGROUND: Children undergoing heart transplant are at higher risk of developing post-transplant lymphoproliferative disorder (PTLD) than other solid organ recipients. The factors driving that risk are unclear. This study investigated risk factors for PTLD in children transplanted at 1 of 2 United Kingdom pediatric cardiac transplantation centers. METHODS: All children (<18 years, n = 200) transplanted at our institution over a 16-year period were analyzed. Freedom from PTLD was assessed using the Kaplan-Meier method and Cox proportional regression. RESULTS: PTLD occurred in 17 of 71 children transplanted for congenital heart disease (CHD) and 18 of 129 transplanted for acquired cardiomyopathy (ACM). The cumulative incidence of all PTLD was 21.1% at 5 years after transplant. Median time from transplant to PTLD was 2.9 years (interquartile range: 0.9-4.6). Negative Epstein-Barr virus (EBV) serostatus pre-transplant (adjusted hazard ratio [HR]: 2.7, 95% CI: 1.3-5.6, p = 0.01) and underlying CHD (adjusted HR: 3.2, 95% CI: 1.4-7.4, p = 0.007) were independently associated with higher risk of PTLD. Age at thymectomy was significantly different between children with CHD and ACM (0.4 vs 5.5 years, p < 0.01). Median CD4+ and CD8+ T lymphocyte counts at 2 years after transplant were significantly lower in children transplanted for CHD vs ACM (CD4+: 391/µl vs 644/µl, p = 0.01; CD8+: 382/µl vs 500/µl, p = 0.01). At 5 years after transplant, those differences persisted among patients who developed PTLD (CD4+, 430/µl vs 963/µl, p < 0.01 and CD8+, 367/µl vs 765/µl, p < 0.01). CONCLUSION: Underlying CHD is an independent risk factor for PTLD and is associated with a younger age at thymectomy. A persistent association with altered T lymphocyte subsets may contribute to the impaired response to primary EBV infection and increase the risk of PTLD.

Do as I say or die: compliance in adolescents with cancer.

Adolescence is a time of great physical change and maturing brain function. This leads to adolescents establishing independence and coming to terms with the implications of their own actions. Not surprisingly, this phase is characterized by experimentation with both constructive and destructive behavior. Studies in many areas of chronic illness have shown that adolescents frequently neglect their care and revolt against the rules established during their childhood. It is therefore to be expected that teenagers diagnosed with a life threatening illness, such as cancer, may on occasion not fully comply with their therapy. The way forward includes improving communication and fully involving these young persons in their treatment planning, thereby moving from compliance to concordance. Additional improvements should be sought in medication, early recognition and support of familial or social problems, and using a specific adolescent multidisciplinary team. Research should not be limited to clinical trials.

Epidemiology of leukaemia and lymphoma in children and young adults from the north of England, 1990-2002.

AIM: We aimed to describe and contrast the epidemiology of haematological malignancies among 0-14 and 15-24-year-olds in northern England from 1990 to 2002 and compare clinical trial entry by age group. PATIENTS AND METHODS: Incidence rates were examined by age, sex and period of diagnosis and differences were tested using Poisson regression. Differences and trends in survival were assessed using Cox regression. RESULTS: 1680 subjects were included comprising 948 leukaemias and 732 lymphomas. Incidence rates for acute lymphoblastic leukaemia were significantly higher for 0-14 compared to 15-24-year-olds, whilst Hodgkin lymphoma showed the reverse. No significant changes in incidence were observed. 60% of leukaemia patients aged 15-24 years entered trials compared to 92% of 0-14-year-olds. Survival rates were significantly lower and improved less markedly over time for 15-24 compared to 0-14-year-olds, particularly for leukaemia. CONCLUSIONS: Trial accrual rates need to be improved amongst 15-24-year-olds and a more structured follow-up approach adopted for this unique population.

Cancer in adolescents and young adults aged 15-24 years: a report from the North of England young person's malignant disease registry, UK.

BACKGROUND: Descriptions of population-based data have rarely been published specifically for adolescents and young adults with cancer. PROCEDURE: Data on young adults (15-24 years) diagnosed with cancer in the North of England from 1968 to 1997 were obtained from the Northern Region Young Person's Malignant Disease Registry. Temporal changes in incidence and survival rates were investigated. RESULTS: There were 2,329 first cancers diagnosed over the study period (M:F 1.22:1). Overall age standardized incidence was 174 cases per million 15-24 years old, per year, 190 for males and 157 for females. The most common cancers in young adults were Hodgkin disease (19%), carcinomas (15%), central nervous system tumors (14%), germ cell tumors (13%), and leukemia (11%). Comparing incidence for 1968-1977 with 1988-1997 there were significant increases in the incidence of bone tumors (rate ratio 1.72, 95% CI 1.10-2.68), testicular tumors (rate ratio 1.64, 95% CI 1.16-2.32), thyroid cancer (rate ratio 2.63, 95% CI 1.37-5.02), and malignant melanoma (rate ratio 2.04, 95% CI 1.36-3.08). Survival rates improved significantly (P < 0.001) over the study period; 5-year survival rates over the three time periods 1968-1977, 1978-1987, 1988-1997 for all cancers were 45% (95% CI 41%-49%), 62% (95% CI 58%-65%), and 74% (95% CI 71%-77%) respectively. CONCLUSIONS: Survival rates improved and there were significant increases in incidence for specific cancers in young adults in the North of England. Further research is required to identify the reasons for changing incidence and to investigate the late effects of treatment among survivors.

Factors influencing early failure of central venous catheters in children with cancer.

BACKGROUND: The authors report the results of a prospective, multicenter, multidisciplinary study of central venous catheters (CVCs) in pediatric oncology patients analyzing factors involved in early failure. METHODS: Information was collected from parent-held records on the fate of 824 devices inserted over a 20-month period, 415 of which were no longer in situ. RESULTS: Within the first 7 weeks after insertion, there were 66 failures, all occurring in external lines. Accidental dislodgement was the principal reason for CVC failure (44 of 66, 67%). Detailed analysis of the reason for failure of this large subgroup showed 11 factors individually associated with early dislodgement, of which, 4 were independently associated with failure by multivariate analysis. These 4 variables were the use of multilumen catheters, the absence of a skin exit site suture, platelet transfusion at the time of insertion, and patient age less than 2 years. CONCLUSIONS: This study confirms the multiple influences on successful CVC usage. Our analysis supports the principle of only using multilumen lines when clinically essential. The findings also support the inception of randomized studies of fixation, particularly in infants.