BJH - volume 11, issue 7, november 2020
J. De Munter , M. Quaghebeur , R. Callens MD, K. Maes MD
In Belgium, over 1,800 adolescents and young adults (AYAs) aged 15–39 are diagnosed annually with cancer. Of all yearly new cancer diagnoses in Belgium, AYA cancers are rare because they are rare in absolute numbers, or because they are rare examples of common cancers occurring outside of the usual age range. Leukaemia and lymphoma’s represent the most common AYA haematological cancers among the AYA population. Apart from the treatment(s) of cancer, the specific needs of young people with haematological malignancies are defined as much, or more, by their age and developmental stage as their life-threatening disease. In June 2018, an AYA interest group under the guidance of “Kom op tegen Kanker” published a blueprint for age-specific care for young people with cancer to highlight the current and future needs of AYA specific cancer care. Current healthcare professional education, training programs and healthcare settings do not address AYA-specific issues. Cure and care is currently exclusively approached from paediatric or adult care perspective. This compartmentalised approach to cancer care can result in a blind spot for AYA comprehensive age developmental cancer care for youngsters and their caregivers. Between the current paediatric and adult silos of care, there is an unmet need for comprehensive AYA cancer care. This care should focus on specific topics to support young people with haematological cancer during treatment, into survivorship care or with early integration of palliative care, providing comprehensive support for AYA patient with limited-life expectations.
(BELG J HEMATOL 2020;11(7):275-81)
Read moreBJH - volume 11, issue 7, november 2020
P. Zachée MD, PhD
Due to intensive travel connections and global warming, it is possible that the vector adapts to the climatic environment of the northern half-front and that an initially tropical infectious disease becomes an emerging infectious disease in European countries. For this reason, we raise the question in the Belgian Hematology Society journal: Is microfilaria a transfusion transmissible disease? The answer is no.
(BELG J HEMATOL 2020;11(7):282-5)
Read moreBJH - volume 11, issue 6, october 2020
L. De Smaele , M. Hofmans MD, PhD, T. Lammens PhD, A. Van Damme MD, PhD, J. van der Werff ten Bosch MD, PhD, A. Ferster MD, PhD, J. Verlooy MD, C. Chantrain , J. Philippé MD, PhD, N. Van Roy PhD, P. De Paepe MD, PhD, V. Labarque MD, PhD, B. De Moerloose MD, PhD
Childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukaemia (JMML) are very rare clonal stem cell disorders of early childhood. Paediatric MDS can be further subdivided in refractory cytopenia of childhood (RCC) and high grade MDS, in case of excess blasts. Given their rarity, little is known about the epidemiology of these diseases in Belgium. The aim of this study is to investigate the incidence, characteristics, treatment and prognosis of paediatric MDS and JMML in Belgium. Prospectively collected data of 56 Belgian patients with MDS and JMML were enrolled in the study, of which 41 (73%) with MDS, eleven with JMML (20%) and four (7%) with Noonan syndrome associated myeloproliferative disorder. The incidence rates of MDS and JMML in Belgium were 1.5 and 0.4 per million children per year respectively, with a median age of diagnosis of 9.3 years for RCC, 9.5 years for high grade MDS and 2.6 years for JMML. Monosomy 7 was the most common cytogenetic abnormality and could particularly be found in high grade MDS (33%) and JMML (45%). RCC treatment consisted of immunosuppressive therapy (IST) and haematopoietic stem cell transplantation (HSCT), but in high grade MDS and JMML only HSCT was a valid treatment option. Overall survival was significantly lower in high grade MDS (45.0%) compared to JMML (79.5%) and RCC (80.6%) (log-rank p-value = 0.038), whereas event-free survival (EFS) was comparably low in high grade MDS and JMML (46.7% and 58.4% respectively) due to a high cumulative incidence of relapse (CIR) of 33% and 29.9%, respectively. Outcome was best for RCC patients with highest EFS (76.3%; 57.1% if IST failure was considered as event) and lowest CIR (9.3%). This study highlights that paediatric MDS and JMML are very rare disorders with associated morbidity and mortality, especially in high grade MDS and JMML. Considering the high relapse risk in high grade MDS and JMML, new therapeutic options are required.
(BELG J HEMATOL 2020;11(6):233-9)
Read moreBJH - volume 11, issue 6, october 2020
C. Tersteeg PhD, H. Deckmyn PhD
Blood platelets are playing a key role in maintaining the integrity of the vascular system or haemostasis, however they can become detrimental when activated at sites of e.g. atherosclerosis, potentially leading to thrombosis or occlusion with devastating effects. To prevent this, a number of antiplatelet agents is currently used in the clinic. However, all antiplatelet agents are accompanied with an increased risk of bleeding, and hence the search for better and safer compounds is ongoing. In this effort, a good understanding of the biochemistry of platelet activation is of primordial importance. In the present review, we intend to bring together the current knowledge on platelet behaviour in thrombosis and haemostasis in a coherent manner by subdividing the entire process into different steps: platelet adhesion, activation, amplification, aggregation, shape change and clot retraction, as well as inhibition of platelet activation and aggregation.
(BELG J HEMATOL 2020;11(6):240-5)
Read moreBJH - volume 11, issue 6, october 2020
B. Depreter PhD, PharmD, B. De Moerloose MD, PhD, J. Philippé MD, PhD, T. Lammens PhD
Ample evidence was provided these past decades that leukaemic stem cells (LSC) play a role in the outcome of adult and paediatric acute myeloid leukaemia (AML) patients. Although it is generally accepted that the CD34+/ CD38- compartment is most LSC-enriched, novel data have emerged illustrating a distinct biology between CD34+ and CD34- AML. In this review, we discuss the main LSC phenotypes in CD34+ and CD34- AML , as they are of utmost importance for the development of broadly applicable LSC-targeted strategies. The leukaemia-initiating capacity of these cells upon xenografting is still considered to be the gold standard for LSC detection. However, more feasible techniques have been researched to allow the implementation of LSC measurements into clinical practice. Here, we summarise the current state-of-the-art methodologies using flow cytometry and molecular detection, and emphasise their relevance in terms of prognosis and targeted drug therapy.
(BELG J HEMATOL 2020;11(6):246-52)
Read moreBJH - volume 11, issue 4, june 2020
J. Versluis MD, PhD, J.J. Cornelissen MD, PhD
The majority of patients with acute myeloid leukaemia (AML) obtain a first complete remission (CR) with intensive induction chemotherapy. Despite post-remission treatment with allogeneic hematopoietic stem cell transplantation (alloSCT), the incidence of relapse remains considerable and depends on the risk of the AML. In addition, the assessment of the quality of CR with measurable residual disease (MRD) has become pivotal in the prognostication of AML patients. MRD may be detected with multiparametric flow cytometry and/or molecular methods including qPCR for specific mutations or next-generation sequencing. Patients with MRD have a high risk of short-term AML recurrence and may benefit from personalised application of post-remission treatment with alloSCT. The graft-versus-leukaemia effect of alloSCT appears to be virtually similar in both MRD positive and MRD negative patients suggesting that alloSCT could be applied not only based on the risk of the disease and quality of remission, but also on the risk of the treatment. Such a risk-adapted approach is recommended for the clinical assessment of all AML patients and should include AML risk, MRD status, and the risk for non-relapse mortality, preferably addressed by dedicated risk scores.
(BELG J HEMATOL 2020;11(4):147–52)
Read moreBJH - volume 11, issue 4, june 2020
M.G.M. Roemer PhD, B. Ylstra PhD, D. de Jong MD, PhD, M.E.D. Chamuleau MD, PhD
Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin lymphoma. The addition of rituximab to the chemotherapy regimen (R-CHOP) has resulted in a significant improvement in survival of DLBCL patients. However, 30–40% of patients still have refractory disease or develop a recurrence. The effectiveness of treatment is limited by the considerable morphological, immunophenotypic, genetic and clinical heterogeneity of DLBCLs. Since 2000, increasing knowledge about the genetic landscape of DLBCL has led to the identification of various genetic prognostic factors, which are associated with poor prognosis following standard R-CHOP treatment, such as ABC genotype and MYC translocation. However, more recent large genomic studies have shown that the genetic landscape of DLBCL is far more complex than this. In this article, these new insights and the first results of prospective clinical studies based on genetic subtypes directed treatment choice will be discussed. This article aims to provide a basis for discussions on future implementation of the knowledge on the genetic complexity of DLBCL in next generation treatment.
(BELG J HEMATOL 2020;11(4):153–8)
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