Articles

Highlights in red blood cell disorders

BJH - volume 11, issue 1, february 2020

V. Labarque MD, PhD

Summary

In the field of red blood cell disorders, ASH 2019 mainly focused on sickle cell disease (SCD). Current disease-modifying therapeutic options for SCD consist of hydroxyurea and chronic transfusions but none of them can fully prevent complications and both have their drawbacks. A haematopoietic stem cell transplantation is the only curative option to date. Yet, frequently a compatible donor is missing. ASH 2019 featured several abstracts on optimising disease-modifying therapies as well as curative options, such as gene therapy. A selection of abstracts is discussed below.

(BELG J HEMATOL 2020;11(1):21–6)

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BHS clinical guidelines on the management of acute complications in sickle cell disease

BJH - volume 10, issue 4, june 2019

T. van Genechten MD, A. Vanderfaeillie MD, M.A. Azerad MD, D. Kieffer PhD, PharmD, V. Labarque MD, PhD, B. Gulbis MD, PhD, A. Ferster MD, PhD, B. De Wilde MD, PhD

With the increasing prevalence of sickle cell disease patients in Western countries, it is of importance to improve awareness among medical doctors of its complications. To reduce long-term morbidity and mortality, the prompt recognition and treatment of acute complications is important. The existing clinical guideline ‘Follow-up and treatment of patients with sickle cell disease hospitalised for Vaso Occlusive Crisis or infection’, published in 2012 by the Belgian Haematological Society, was revisited to better suit the practical needs of first-line practitioners.

(BELG J HEMATOL 2019;10(4):165–8)

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O09 VISUALIZING DENSE GRANULE FORMATION IN MEGAKARYOCYTES USING ADVANCED MICROSCOPY AND ITS APPLICATION FOR HERMANSKY-PUDLAK SYNDROME

BJH - 2019, issue ?, february 2019

J. Heremans , C. Thys , K. Cludts , V. Labarque MD, PhD, C. Van Geet , K. Freson

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Retrospective study of infant leukaemia in the University Hospitals of Leuven and Ghent

BJH - volume 9, issue 2, march 2018

B. De Moerloose MD, PhD, E. Nauwynck MD, K. Arts MD, L. Willems MD, PhD, V. Labarque MD, PhD, T. Lammens PhD, A. Uyttebroeck MD, PhD

SUMMARY

Infant leukaemia is a rare disease but the 3rd most frequent malignancy in this age group. Both acute lymphoblastic leukaemia and acute myeloid leukaemia in the first year of life have particular clinical and biological characteristics such as B-cell phenotype with co-expression of myeloid markers in acute lymphoblastic leukaemia, FAB M5 or M7 in acute myeloid leukaemia, the presence of extramedullary symptoms and a high frequency of KMT2A rearrangements. Survival rates for infant acute leukaemia are worse than for older children. In this study, the characteristics and outcome of 50 infants with acute lymphoblastic leukaemia and acute myeloid leukaemia treated at the University Hospitals of Ghent and Leuven between 1989 and 2015 were studied and correlated with literature data. With event-free survival and overall survival rates of 44% and 52% for the entire cohort, the outcome of these patients was comparable to those in published clinical trials. In general, the event-free survival and overall survival was superior in acute myeloid leukaemia compared to acute lymphoblastic leukaemia infants and not influenced by age (< or ≥6 months), white blood cell count at diagnosis or presence of a KMT2A rearrangement. For future trials in infant leukaemia, the high number of early deaths, toxic deaths and relapses remain the most challenging problems.

(BELG J HEMATOL 2018;9(2):57–63.)

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Belgian Society of Paediatric Haematology Oncology symposium

BJH - volume 5, issue 2, june 2014

V. Labarque MD, PhD

A summary of the BSPHO program hosted by the general annual meeting of the BHS in Ghent on Friday, January 31st, 2014.

(BELG J HEMATOL 2014;5(2):68–71)

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

BJH - volume 3, issue 1, march 2012

N. Reynaert MD, V. Labarque MD, PhD, A. Uyttebroeck MD, PhD, E. Levtchenko MD, PhD, M. Renard

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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