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.

Germline TET2 loss of function causes childhood immunodeficiency and lymphoma.

Molecular dissection of inborn errors of immunity can help to elucidate the nonredundant functions of individual genes. We studied 3 children with an immune dysregulation syndrome of susceptibility to infection, lymphadenopathy, hepatosplenomegaly, developmental delay, autoimmunity, and lymphoma of B-cell (n = 2) or T-cell (n = 1) origin. All 3 showed early autologous T-cell reconstitution following allogeneic hematopoietic stem cell transplantation.

The use of anthracyclines in the treatment of endemic Burkitt lymphoma.

Burkitt lymphoma is the most common malignancy in children in Malawi, the world's poorest country, where there is a long history of treating this disease using a 28-day cyclophosphamide-based protocol. Stage III/IV disease has had poor outcomes. In an attempt to improve the outcome for higher stage disease, anthracyclines were added to the existing protocol.

Langerhans cell histiocytosis in children with congenital anomalies: a population-based record linkage study.

Background: The etiology of Langerhans cell histiocytosis (LCH), a rare cancer-like disorder of the immune system, is largely unknown although a genetic component has been suggested based on familial cases, and reports of chromosome instability and genetic mutation. Associations between various cancers and congenital anomalies have been reported and although congenital anomalies have been noted in children with LCH only one study to date has reported their frequency. An association between congenital anomalies and LCH may suggest a common etiological pathway, in particular, a genetic pathway.

Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood.

Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty-eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO-) risk, were evaluated for bone lesions at diagnosis.

Cutaneous B-lymphoblastic lymphoma with IL3/IgH translocation presenting with hypereosinophilia and acute endocarditis.

Hypereosinophilia is a rare phenomenon associated with childhood malignancy, predominantly acute lymphoblastic leukaemia. Causation is unclear and likely to have multiple mechanisms. We report a six year old boy presenting with hypereosinophilia and associated Loeffler endocarditis.

Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH)-III tested risk-adjusted, intensified, longer treatment of multisystem LCH (MS-LCH), for which optimal therapy has been elusive. Stratified by risk organ involvement (high [RO+] or low [RO-] risk groups), > 400 patients were randomized. RO+ patients received 1 to 2 six-week courses of vinblastine+prednisone (Arm A) or vinblastine + prednisone + methotrexate (Arm B).

Langerhans cell histiocytosis: a multisystem disorder.

Langerhans cell histiocytosis can involve single or multiple organ/tissue systems and may go undiagnosed for years until it enters the clinician's differential diagnosis framework. We report on a young patient who initially presented with diabetes insipidus and subsequently with pyrexia of unknown origin. She progressed from single system Langerhans cell histiocytosis to multisystem involvement and remains in long-term remission following chemotherapy.

Post cardiac transplantation lymphoproliferative disorder presenting as t(8;14) Burkitt leukaemia/lymphoma treated with low intensity chemotherapy and rituximab.

Background: Post-transplant lymphoproliferative disorder (PTLD) occasionally presents as Burkitt lymphoma/L3 leukaemia (BLL).

Procedure: We reviewed records of cases of PTLD post-cardiac transplantation (1990-2007) occurring in our unit.

Results: There were 15 episodes in 13 patients including four cases of EBV-driven Burkitt-type disease with t(8;14) translocations presenting with advanced stage disease.

Langerhans cell histiocytosis.

Langerhans cell histiocytosis is a rare disease. Depending on which organs are involved, the disease may prove rapidly fatal, develop a chronic reactivating but therapy-responsive pattern or resolve spontaneously. Understanding of the pathology of the disease is progressing rapidly, and while clinical trials of standard chemotherapy agents continue, it is likely that novel targeted therapy will become feasible in the next decade.

Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland.

Objectives: There are few published studies on the epidemiology of Langerhans cell histiocytosis (LCH). We undertook a survey to ascertain all newly diagnosed cases aged 0-16 years in the UK and Republic of Ireland.

Design: Three methods of ascertainment were used: the British Paediatric Surveillance Unit (BPSU) system, a survey by Newcastle University, and the Children's Cancer and Leukaemia Group (CCLG) registry.

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.

Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry.

Objective: To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality.

Study Design: Retrospective analysis of 335 patients with MS-LCH and documented complete disease resolution (NAD1).

Results: The probability of a reactivation within 5 years of NAD1 was 46%.

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.

Outcome of a risk-related therapeutic strategy used prospectively in a population-based study of Hodgkin's lymphoma in adolescents.

The aim was to assess outcome in a population-based cohort of adolescents with Hodgkin's lymphoma (HL) diagnosed in the UK's northern region over a 10-year period. Among a population of 3.09 million, 55 of 676 patients (8%) diagnosed with HL were aged 13-19.

Juvenile xanthogranuloma associated with contralateral lymphadenopathy.

Juvenile xanthogranuloma is an uncommon, benign histiocytic condition, primarily affecting children less than 1 year of age. Although usually only cutaneous lesions are found, systemic manifestations of the disease have been reported. We present a child with juvenile xanthogranuloma of the right cheek associated with contralateral cervical lymph node histiocytic infiltration.

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.

Follicular dendritic cell tumour in a 9-year-old child.

Follicular dendritic cell tumour (FDCT) or sarcoma is a rare tumour first described in 1986. Some 80 cases have been reported, the youngest being in teenagers. Our patient first presented at 9 years of age with a cervical mass that was removed and revealed an apparently benign, but florid reactive process.

A comparison of self-reported satisfaction between adolescents treated in a "teenage" unit with those treated in adult or paediatric units.

Background: Despite recommendations that adolescents should have in-patient management amongst their peers, there is little literature to support this. The study aim was to evaluate and contrast patient satisfaction for teenage cancer patients treated in two settings. The first is a split site unit (a paediatric ward and adult cancer centre in different locations within one city) and the second, a dedicated adolescent unit for patients aged 13-20.

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.

Starting an adolescent cancer unit: why does it take so long?

A pragmatic approach is discussed based on the authors' differing experiences while designing new units specifically for adolescents with cancer in Leeds and Newcastle upon Tyne. Planning and implementation are complex and very time consuming. Areas to be considered include: formally identifying needs and adapting to existing local circumstances, convening a working group and involving potential stakeholders at an early stage, designing a suitable physical space, recruiting and integrating staff to create a supportive environment, obtaining financial support, and developing operational policies.

Demographic study of leukaemia presenting within the first 3 months of life in the Northern Health Region of England.

Aims: To determine the incidence and outcome of congenital leukaemia.

Methods: Retrospective population based study of putative leukaemia arising during the first 3 months of life over an 18 year period within the Northern Health Region of England.

Results: Nine infants with putative leukaemia were identified.