B. Gulbis MD, PhD

Highlights in red blood cell disorders

SUMMARY

Research in the field of haemoglobinopathies, and in particular for sickle cell disease, has enabled the implementation of new therapies, both modifying and curative. The congress was the opportunity to present the experience gained from their use in real life or in phase III trials; it has demonstrated their potential benefit while emphasising that careful monitoring of these new treatments remains necessary. The congress was also an opportunity to present new tools used in the field of haemoglobinopathies. They contribute to the assessment of the impact of new modifying therapies in sickle cell disease, i.e., oxygen gradient ektacytometry, or in the field of diagnosis using artificial intelligence, i.e., SCD silent cerebral infarct and liver iron overload.

(BELG J HEMATOL 2022;13(5):207–9)

Highlights in red blood cell disorders

SUMMARY

As the COVID-19 pandemic is a challenge for all of us, the EHA meeting was the occasion to exchange experiences about initiatives developed with the aim of improving the management of patients suffering from a pathology of the red blood cell and infected with COVID-19. It was also an opportunity to present the developments observed in clinical studies involving new therapies or the combination of therapies, or to get an overview of the evolutions in the field of red blood cell diseases.

(BELG J HEMATOL 2020;11(5):226-9)

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

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)

Highlights in the field of red blood cell disorders

SUMMARY

Red blood cell pathologies were highlighted during the congress. In particular, the Topics-in-Focus program put the spotlight on haemoglobinopathies. As in oncology, these heterogeneous (very) rare diseases also benefit from new tools for their diagnosis and from new or evolving therapies. Gene therapy is probably the most exciting one as it represents a curative approach.

(BELG J HEMATOL 2018;9(4):161–3)

PP2.5 Hypercoagulability in adult sickle cell patients as compared to sickle cell trait subjects and a control group

Epidemiological data on sickle cell disease in Belgium

summary

Although neonatal screening for sickle cell disease is one of the best tools for reducing mortality during infancy and early childhood, it is not part of the approved neonatal screening programme in Belgium. As epidemiological data on sickle cell disease are still incomplete in Belgium, we planned to screen the samples of newborns available in the biggest reference centre for approved neonatal screening in Flanders. From July to December 2013, a total of 18,989 newborns from 36 Flemish maternity wards were systematically screened, representing about 60% of the total number of births in Flanders. For the same period, results of the neonatal screening that is routinely performed for sickle cell disease in three other Belgian centres were collected. Overall, 39,599 newborns were screened, representing about two-thirds of Belgian births for this period. With an incidence of sickle cell disease and sickle cell trait of 1/2,329 and 1/77, respectively, sickle cell disease is the most frequently inherited disease observed in the population tested; the highest incidences were registered in urban areas. In addition, screening techniques identified 122 other clinically significant haemoglobin (Hb) variant carriers (83 for HbC, twenty for HbE, thirteen for HbD-Punjab, and six for HbO-Arab) and two HbC diseases. Carriers of clinically significant Hb variants were observed in almost all the maternity wards included in the study, showing a wide dispersal of populations at risk. These epidemiological data remind us of the warnings and recommendations from the World Health Organization, urging policy-makers to consider the most appropriate strategy to prevent and treat patients with sickle cell disease in Belgium.

(BELG J HEMATOL 2015;6(4): 135–41)

Thrombin generation in the plasma of sickle cell trait adults shows prothrombotic profile as compared to normal adults