OBJECTIVE: In Australia acute idiopathic thrombocytopenic purpura (ITP) is mainly treated by paediatricians (either general paediatricians or paediatric haematologists/oncologists). A survey was conducted to gauge the current practice of treating children with acute ITP in Australia. METHODS: All practising Australian paediatricians registered by the Royal Australasian College of Physicians were surveyed regarding their intended management of children with acute ITP. The questionnaire, adapted from a study of paediatric haematologists/oncologists in North America, presented four clinical scenarios of children with acute ITP with a platelet count of 3000 x 10(9)/L, with and without mucosal bleeding (wet and dry purpura, respectively). Questionnaires were returned by mail or filled in online at a dedicated webpage. RESULTS: Five hundred and sixty-three of 1097 (51%) paediatricians responded to the survey. Data from 140 who had treated at least one child with ITP in the previous 12 months were analysed. Respondents indicated that children with acute ITP are usually or always hospitalised (58-92%) and that 48% would be given active treatment, even with dry purpura. Various regimens of i.v. immunoglobulin or corticosteroids are used when treatment is administered. In comparing Australian and North American management of acute ITP there were many similarities, although Australian paediatricians were less likely to arrange a bone marrow aspirate if corticosteroids were prescribed. CONCLUSIONS: There is great variation in the intended management of children with acute ITP in Australia. Previously published management recommendations regarding investigation and treatment have had little impact on intended practice. Prospective studies are required to evaluate hypotheses so as to produce evidence-based recommendations for treatment of patients with acute ITP.

Acute Disease↗

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Reduction of Factor VIII activity in cryoprecipitate obtained from acidified plasma.

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Acids↗

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Neurobehavioural sequelae following cranial irradiation and chemotherapy in children: an analysis of risk factors.

Neurobehavioural deficits are commonly reported following treatment for childhood cancers. This study examined the impact of cranial irradiation (CRT) and chemotherapy in children, aiming to identify factors detrimental to long-term outcome. The study compared survivors of acute lymphoblastic leukemia (ALL), treated with CRT and chemotherapy (CRT group: n = 100), survivors of cancers treated with chemotherapy only (n = 50) and healthy controls (n = 100) for intelligence, academic achievement, information processing, learning, and executive function. CRT and chemotherapy in combination were associated with reduced intelligence, educational skill, immediate memory, processing speed, and executive function. Children treated with chemotherapy alone exhibited subtle information processing deficits. Within the CRT group, younger age at treatment was predictive of deficits in non-verbal ability, educational skills and executive functions. High dose CRT was associated with poorer information processing and lower arithmetic ability.

Achievement↗

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Coexistent T-cell lymphoblastic lymphoma and an atypical myeloproliferative disorder associated with t(8;13)(p21;q14).

This report describes a neoplasm exhibiting both lymphoid and myeloid differentiation associated with an acquired balanced translocation between chromosomes 8 and 13 occurring in a 10-year-old boy. Serial lymph node biopsies revealed the presence of both lymphoblastic lymphoma and an atypical myeloproliferative disorder within the same node. Immunophenotyping was consistent with the presence of an immature T-cell population within the nodal biopsy specimens. Cytogenetic analysis of the bone marrow and lymph node biopsy specimens revealed a unique translocation, t(8;13) (p21;q14). Molecular analysis revealed rearrangement of the immunoglobulin heavy chain gene and germline configuration of the T-cell receptor gene. The patient had a poor response to classical T-cell acute lymphocytic leukemia/lymphoma therapy and was changed to a myeloid leukemia protocol with good response. He underwent bone marrow transplantation but died soon after of overwhelming graft-versus-host disease. We found five similar cases in the literature, suggesting the existence of a subset of mixed lymphoid/myeloid disorders with 8p;13q translocations, in which the clinical picture is dictated by the myeloid element.

Child↗