Australian and New Zealand consensus guideline for paediatric newly diagnosed immune thrombocytopaenia endorsed by Australian New Zealand Children's Haematology and Oncology Group.

In children, the majority of cases are self-limiting and thus many paediatric patients can be managed conservatively with minimal complications. This varies considerably compared to adult newly diagnosed immune thrombocytopaenia (NDITP) where, in most cases, thrombocytopaenia persists with higher risk of moderate to severe bleeding complications. In the past decade, local and international guidelines have emerged to support approaches to the investigation and management of NDITP, with a focus primarily on adult immune thrombocytopaenia (ITP). International consensus guidelines on paediatric NDITP have been developed, however gaps remain, and approaches vary between North American, Asia, Europe and the UK. There are no current Australian or New Zealand paediatric ITP guidelines readily available, rather differing guidelines for each state, territory or island. These inconsistencies cause uncertainty for patients, families and physicians managing cases. Subsequently, physicians, including paediatric haematologists and general paediatricians, have come together to provide a consensus approach guideline specific to paediatric NDITP for Australian or New Zealand. Persistent or chronic paediatric ITP remains a complex and separate entity and are not discussed here.

Childhood cancer registration in New Zealand: A registry collaboration to assess and improve data quality.

AIM: To evaluate the completeness and accuracy of child cancer registration in New Zealand. METHODS: Registrations for children aged 0-14 diagnosed between 1/1/2010 and 31/12/2014 were obtained from the New Zealand Cancer Registry (NZCR) and the New Zealand Children's Cancer Registry (NZCCR). Six key data fields were matched using National Health Index numbers in order to identify and resolve registration discrepancies. Capture-recapture methods were used to assess the completeness of cancer registration. RESULTS: 794 unique cases were reported; 718 from the NZCR, 721 from the NZCCR and 643 from both registries. 27 invalid cancer registrations were identified, including 19 residents of the Pacific Islands who had travelled to New Zealand for treatment. The NZCCR provided 55 non-malignant central nervous system tumour and 16 Langerhans cell histiocytosis cases which were not registered by the NZCR. The NZCR alerted the NZCCR to 18 cases missed due to human error and 23 cases that had not been referred to the specialist paediatric oncology centres. 762 cases were verified as true incident cases, an incidence rate of 166.8 per million. Registration accuracy for six key data fields was 98.6%. According to their respective inclusion criteria case completeness was 99.3% for the NZCR and 94.4% for the NZCCR. For childhood malignancies covered by both registries, capture-recapture methods estimated case ascertainment at greater than 99.9%. CONCLUSION: With two national registries covering childhood cancers, New Zealand is uniquely positioned to undertake regular cooperative activities to ensure high quality data is available for research and patient care.

Imatinib mesylate-induced pseudoporphyria in two children.

Imatinib mesylate was the first of several tyrosine kinase inhibitors approved for use in the treatment of a number of human cancers. Adverse cutaneous reactions to imatinib are common. Pseudoporphyria has been infrequently reported in adults undergoing imatinib therapy for chronic myeloid leukemia. We present two children with pseudoporphyria induced by imatinib therapy for hematologic malignancies. In view of the burgeoning use of imatinib in children, physicians should be aware that pseudoporphyria may develop as a consequence of imatinib therapy.

Enrollment in clinical cancer trials: how are we doing and what are the obstacles to improving enrollment rates? A 2-year retrospective review of pediatric cancer trial enrollment in New Zealand.

Clinical trials contribute to the establishment of the best therapy for children with cancer. This study looks at rates of enrollment in therapeutic clinical trials over a 2-year period in New Zealand and examines the reasons for nonenrollment. All new diagnoses of cancer in children aged 16 or younger over the period of 1 January, 2009 to 31 December, 2010 were identified through the New Zealand Child Cancer Registry. Clinical trial enrollment status was identified from the medical records. For those not enrolled, the reason for nonenrollment was ascertained. A total of 28% of children diagnosed with cancer who received chemotherapy with curative intent in this time period were enrolled on clinical trials. The 2 most common reasons for nonenrollment in this study were that no study was open locally in which to enroll children (27%) or that previously open-clinical trials were closed to accrual at the time of the child's diagnosis (20%). In New Zealand, enrollment rates on clinical trials for children with cancer are lower than expected.

Testing for heritable thrombophilia in children at Starship Children's Hospital: an audit of requests between 2004 and 2009.

AIMS: The aim of this study was to review patterns of requests for heritable thrombophilia and to audit these findings against an international standard. METHODS: Review of requests for antithrombin, protein C, protein S, activated protein C resistance, Factor V Leiden and prothrombin G20210A mutation analysis in children <16 years between 1/1/2004 and 31/12/2009. Data for patient characteristics, test results, origin of request, requesting department and indication were obtained. The 2010 British Committee for Standards in Haematology (BCSH) clinical guidelines for testing for heritable thrombophilia was used as the standard for the audit. RESULTS: On 269 patients, 379 requests were made. Thirty-four per cent of tests were abnormal but only 36% of abnormal tests were repeated. Seven per cent of patients were confirmed with a heritable thrombophilia. Thirty-four tests were performed on patients on anticoagulation. The median age was 6.903 years. Fifty-three per cent of requests came from a ward, 28% from outpatients, 14% from an intensive care department and 5% from the emergency department. Departments most frequently requesting tests were neurology (20%), paediatric intensive care (15%) and cardiology (12%). Indications for testing were arterial thrombosis (5%), cerebral vein thrombosis (4%), deep vein thrombosis (12%), stroke (23%), asymptomatic relative (6%), intra-abdominal vein thrombosis (8%), start oestrogen containing medication (2%), purpura fulminans (0.2%), heparin resistance (8%) and other (35%). DISCUSSION: The large majority of requests did not satisfy the BCSH criteria. Requesting behaviours are haphazard. Better appreciation of the difficulties of interpreting results in children of different ages and in different clinical settings amongst paediatricians is required.