REVIEW HEMATOLOGY

BCMA targeting in multiple myeloma

SUMMARY

Over the past decade, significant progress was made in the treatment of patients with multiple myeloma (MM). Nevertheless, research efforts continue in an attempt to develop treatment options with novel mechanisms of action that have higher efficacy, can evade resistance to prior lines of treatment and are well tolerated. As the B-cell maturation antigen (BCMA) is preferentially expressed by mature B-lymphocytes and is overexpressed in MM patients, it provides an interesting therapeutic target in MM. Thus far, three treatment modalities have been developed for BCMA targeting; bispecific antibody constructs, antibody-drug conjugates and chimeric antigen receptor (CAR) T-cell therapy, each with its own advantages and challenges. This review provides an overview of the (preliminary) clinical data that were generated with these different treatment modalities.

(BELG J HEMATOL 2020;11(8):387-97)

State of the Art I: Venous thromboembolism

The first state of the art session of the 28^th annual BSTH meeting focussed on venous thromboembolism and was moderated by Alain Gadisseur and Kristel Vandenbosch. Prof. P-YLeRoux (physician in the department of Nuclear Medicine at the Brest University Hospital, France, and senior scientist in the Thrombosis Study group of Western Brittany) opened this session with a talk on the diagnosis of acute pulmonary embolism. Following up on this presentation, prof. dr. S. Konstantinides (Professor for Clinical Trials and Medical director of the multidisciplinary centre for thrombosis and haemostasis (CTH) at the University of Mainz, Germany and professor of Cardiology at the Democritus University of Thrace, Greece), turned the attention to the clinical management of pulmonary embolism.

State of the Art II: Immunothrombosis

The second state of the art session of the 2020 annual BSTH meeting focused on Immunothrombosis. First of all, Prof Adam Cunningham (University of Birmingham, UK) addressed the association between Salmonella infection and thrombosis, after which Assistant Prof. Kimberly Martinod (Catholic University Leuven, Belgium) discussed the role of neutrophil extracellular traps at the interface of thrombosis and inflammation. In a third and final lecture of the session, Dr. Frantzeska Frantzeskaki (Attikon University Hospital, NK University of Athens, Greece) turned the attention to immunothrombosis in patients with acute respiratory distress syndrome.

Adolescents/young adults and their caregiver on the onco-haematology ward

SUMMARY

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)

Is microfilaria a transfusion transmissible disease?

SUMMARY

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)

Retrospective analysis of the incidence and characteristics of paediatric myelodysplastic syndrome and juvenile myelomonocytic leukaemia in Belgium

SUMMARY

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)

The biochemistry of platelet function regulation

SUMMARY

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)