Splenectomy in children with idiopathic thrombocytopenic purpura: A prospective study of 134 children from the Intercontinental Childhood ITP Study Group

BACKGROUND: Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. PROCEDURE: Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in 25 countries participated in a splenectomy registry. Data from 153 patients were submitted, of whom 134 had a splenectomy and were analyzed. RESULTS: The median age at splenectomy was 11.8 (2.7-20.7) years. The median postsplenectomy follow-up was 2.0 (0.1-4.5) years. Pre-splenectomy vaccination was not administered in 21 children (15.7%). Open and laparoscopic splenectomy procedures were performed in 67 and 65 evaluable children, respectively. Surgical technique was not reported in two children. Overall immediate platelet response to splenectomy was achieved in 113 patients (86.3%). Eighty percent of responders maintained their status of response during the following 4 years. Older age, longer duration of ITP, and male gender correlated with a complete response. Post-splenectomy sepsis was reported in seven patients without lethal outcome, although sepsis might be differently defined at participating institutions. CONCLUSIONS: Splenectomy is effective in children with ITP. Management varies greatly in different institutions. These Registry data may serve as a basis for future clinical trials to assess the indication and timing of splenectomy.     

Second primary neoplasms in survivors of Wilms' tumour--a population-based cohort study from the British Childhood Cancer Survivor Study

A British population-based cohort study was carried out to determine the risk of second primary neoplasms in survivors of Wilms' tumour. The cohort was obtained from the British Childhood Cancer Survivor Study, a population-based cohort study of treatment toxicities in 18,044 individuals diagnosed with childhood cancer, at an age of less than 15 years, between 1940 and 1991 in Britain. There were 1,441 Wilms' tumour survivors in the cohort: 732 males (50.8%) and 709 females (49.2%). Total follow-up from 5-year survival was 27,841 person years, mean follow-up of 19.3 years per survivor. There were 81 second primary neoplasms, including 52 solid neoplasms, 3 acute myeloid leukaemias and 26 basal cell carcinomas. Thirty-five of the 39 solid neoplasms that developed in the thoracic, abdominal or pelvic region occurred within irradiated tissue. The standardised incidence ratio for all solid second primary neoplasms was 6.7 (95% CI: 5.0-8.8). Cumulative incidence for all solid second primary neoplasms by ages 30, 40 and 50 years was 2.3% (1.4-3.5%), 6.8% (4.6-9.5%) and 12.2% (7.3-18.4%). The overall risk of second primary neoplasms in survivors of Wilms' tumour treated between 1940 and 1991 was substantial, and solid second tumours tended to develop in the irradiated tissue. Continued follow-up of these survivors is important to monitor such late effects of treatment. It is also important to evaluate the risk of second primary neoplasms following more recent lower radiation dose treatment practices.     

Exposure to power frequency electric fields and the risk of childhood cancer in the UK

The United Kingdom Childhood Cancer Study, a population-based case-control study covering the whole of Great Britain, incorporated a pilot study measuring electric fields. Measurements were made in the homes of 473 children who were diagnosed with a malignant neoplasm between 1992 and 1996 and who were aged 0-14 at diagnosis, together with 453 controls matched on age, sex and geographical location. Exposure assessments comprised resultant spot measurements in the child's bedroom and the family living-room. Temporal stability of bedroom fields was investigated through continuous logging of the 48-h vertical component at the child's bedside supported by repeat spot measurements. The principal exposure metric used was the mean of the pillow and bed centre measurements. For the 273 cases and 276 controls with fully validated measures, comparing those with a measured electric field exposure >/=20 V m(-1) to those in a reference category of exposure <10 V m(-1), odds ratios of 1.31 (95% confidence interval 0.68-2.54) for acute lymphoblastic leukaemia, 1.32 (95% confidence interval 0.73-2.39) for total leukaemia, 2.12 (95% confidence interval 0.78-5.78) for central nervous system cancers and 1.26 (95% confidence interval 0.77-2.07) for all malignancies were obtained. When considering the 426 cases and 419 controls with no invalid measures, the corresponding odds ratios were 0.86 (95% confidence interval 0.49-1.51) for acute lymphoblastic leukaemia, 0.93 (95% confidence interval 0.56-1.54) for total leukaemia, 1.43 (95% confidence interval 0.68-3.02) for central nervous system cancers and 0.90 (95% confidence interval 0.59-1.35) for all malignancies. With exposure modelled as a continuous variable, odds ratios for an increase in the principal metric of 10 V m(-1) were close to unity for all disease categories, never differing significantly from one.     

Childhood and adolescent lymphoma in Spain: incidence and survival trends over 20 years

Background

Lymphoma is the third most common malignancy in children (0–14 years) and the first in adolescents (15–19 years). This population-based study—the largest ever done in Spain—analyses incidence and survival of lymphomas among Spanish children and adolescents.

Patients and methods

1664 lymphoma cases (1983–2007) for incidence and 1030 for survival (1991–2005) followed until 31/12/2010, were provided by 11 cancer registries. Age-adjusted incidence rates (ASRw) to the world standard population were obtained; incidence trends were modelled using the Joinpoint programme, observed survival (OS) was estimated with Kaplan–Meier and trends tested with a log-rank test. Results are presented according to the International Classification of Childhood Cancer-3.

Results

In Spain, the ASRw_0–14 for lymphomas was 17.5 per 1.000.000 child-years and 50.0 the specific rate for adolescents. Overall incidence increased significantly during 1983–1997 with no increases thereafter. Patients over 9 years old showed significant rising trends for all subtypes, except for Burkitt lymphoma (BL) in adolescents. During 2001–2005 (age 0–19 years), 5-year OS was 94 (90–98), 73 (64–83) and 86 (78–94) for Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL) and BL, respectively. No improvement in survival was found. The incidence in Spain was higher than overall European rates, but within the range of that in Southern Europe. Comparing OS in Spain 1991–1995 and 2001–2005 with results for Europe of the Automated Childhood Cancer Information System (ACCIS) (1988–1997) and the European cancer registry-based study on survival and care of cancer patients (EUROCARE) (2000–2007), it was similar for HL and lower for NHL and BL.

Conclusions

Systematic monitoring and analysis of lymphoma paediatric data would provide clinical and epidemiological information to improve the health care of these patients and the outcomes for these malignancies in Spain.

     

Employment status and occupational level of adult survivors of childhood cancer in Great Britain: The British childhood cancer survivor study

The British Childhood Cancer Survivor Study (BCCSS) provides the first detailed investigation of employment and occupation to be undertaken in a large population‐based cohort. Previous studies have been limited by design issues such as using small numbers of survivors with specific diagnoses, and involved limited assessment of employment status and occupational level. The BCCSS includes 17,981 5‐year survivors of childhood cancer. Employment status and occupational level were ascertained by questionnaire from eligible survivors (n = 14,836). Multivariate logistic regression was used to explore factors associated with employment and occupation, and to compare survivors to their demographic peers in the general population. Employment status was available for 10,257 survivors. Gender, current age, cancer type, radiotherapy, age at diagnosis and epilepsy were consistently associated with being: employed; unable to work; in managerial or non‐manual occupations. Overall, survivors were less likely to be working than expected (OR (99% CI): 0.89 (0.81–0.98)), and this deficit was greatest for irradiated CNS neoplasm survivors (0.34 (0.28–0.41)). Compared to the general population, survivors were fivefold more likely to be unable to work due to illness/disability; the excess was 15‐fold among CNS neoplasm survivors treated with radiotherapy. Overall survivors were less likely to be in managerial occupations than expected (0.85 (0.77–0.94)). However, bone sarcoma survivors were more likely to be in these occupations than expected (1.37 (1.01–1.85)) and also similarly for non‐manual occupations (1.90 (1.37–2.62)). Survivors of retinoblastoma (1.55 (1.20–2.01)) and ‘other’ neoplasm group (1.62 (1.30–2.03)) were also more likely to be in non‐manual occupations than expected.