M. Renard

P.71 Swachman-Diamond Syndrome: Frequent misdiagnosis as Jeune Syndrome and other peculiarities

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Less veno-occlusive disease after intravenous versus oral busulfan for autologous haematopoietic stem cell transplantation: the Belgian paediatric experience

Summary

Busulfan is commonly used in preparative conditioning regimens prior to haematopoietic stem cell transplantation in children and young adults for malignant and non-malignant disorders. For many years busulfan was only available in oral form, resulting in large inter- and intra-patients variability in plasma exposure, associated with higher graft failure rate as well as higher toxicity such as veno-occlusive disease. With the development of an intravenous formulation of busulfan, a more accurate control of both the inter- and intra-patient variability has been provided. The goal of this study was to evaluate the use and efficacy of intravenous busulfan in comparison with the oral formulation in children undergoing an autologous transplantation after conditioning with busulfan. Despite the small number of patients, this study confirmed the apparent benefit of intravenous busulfan in children undergoing an autologous HSCT. The use of a five-level dose schedule defined by body weight resulted in an efficient engrafitment with marked reduction in the incidence of veno-occlusive disease compared with oral busulfan. In terms of disease-free outcome, survival and event-free survival, similar results have been obtained in both groups. The choice of this formulation of busulfan should therefore be considered.

(BELG J HEMATOL 2012;3:34–40)

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Pseudohyperkalaemia in T-ALL: how to avoid life-threatening hypokalaemia

Summary

Tumour lysis syndrome is a well-known life-threatening complication in children with acute leukaemia and hyperleukocytosis. It is characterised by hyperkalaemia but it should be distinguished from pseudohyperkalaemia. Various underlying factors for this phenomenon of pseudohyperkalaemia have been suggested. Here, we describe two children with T-cell acute lymphoblastic leukaemia who presented with hyperleukocytosis and hyperkalaemia, in whom the diagnosis of pseudohyperkalaemia was made. We demonstrate that in extreme leukocytosis the use of a vacuum system, pneumatic transport, and sample centrifugation contribute all together to pseudohyperkalaemia. As the leukocyte count decreases, plasma potassium levels are more reliable. Based on our results we suggest to measure potassium in a whole blood sample immediately brought to the laboratory for minimising false results due to ex vivo cell lysis.

(BELG J HEMATOL 2012;3:12–6)

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