Articles

Updated BHS guidelines for the treatment of diffuse large B-cell lymphoma (DLBCL): Novelties anno 2024

BJH - volume 15, issue 4, june 2024

J. Brijs MD, M. André MD, PhD, S. Bailly MD, K. Beel MD, PhD, C. Bonnet MD, G. Crochet MD, P. De Paepe MD, PhD, D. Dierickx MD, PhD, C. Jacquy MD, PhD, K. Saevels MD, S. Snauwaert MD, PhD, E. Van den Neste MD, PhD, V. Vergote MD

SUMMARY

Diffuse large B-cell lymphoma (DLBCL) is an aggressive B-cell lymphoma and represents the most common subtype of B-cell non-Hodgkin lymphomas. The majority of patients (60–70%) can nowadays be cured with first line chemo-immunotherapy (CIT), mostly a combination of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP). The remaining 30–40% of patients with relapsing or refractory (R/R) disease have an unfavourable prognosis. Until recently, these patients could only be cured with platinum-based salvage CIT followed by high-dose chemotherapy and an autologous stem cell transplantation, but with rather disappointing outcomes. However, new and promising treatments for these patients have now found their way into clinical practice, with good response and survival rates and manageable toxicity rates. This article will briefly review the latest advances in the treatment of DLBCL in Belgium, both for newly diagnosed disease and for R/R disease. We will focus on the role of polatuzumab vedotin in first line, chimeric antigen receptor (CAR) T-cell therapy in second line, tafasitamab-lenalidomide in second line or higher, and bispecific antibodies in third line or higher. New treatment algorithms, both for untreated and for R/R DLBCL, clinically oriented and adapted to the Belgian reimbursement criteria, are also presented.

(BELG J HEMATOL 2024;15(4):147–57)

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

BJH - 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

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)

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P.26 Waldenström’s macroglobulinemia and renal insufficiency: a case report

BJH - volume 11, issue Abstract Book BHS, february 2020

S. Snauwaert MD, PhD, T. Lodewyck MD, P. De Paepe MD, PhD

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O.2 Analysis of Phenotype and Outcome in Essential Thrombocythemia with CALR and JAK2 mutations

BJH - volume 6, issue Abstract Book BHS, january 2015

C. AL Assaf , F. Van Obbergh MD, J. Billiet MD, E. Lierman PhD, T. Devos MD, PhD, C. Graux MD, PhD, A.S. Hervent , T. Tousseyn MD, PhD, P. De Paepe MD, PhD, P. Papadopoulos , L. Michaux MD, PhD, P. Vandenberghe MD, PhD

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Juvenile myelomonocytic leukaemia: the quest for more specific therapies

BJH - volume 5, issue 4, december 2014

H. Helsmoortel PhD, T. Lammens PhD, N. Van Roy PhD, J. Philippé MD, PhD, P. De Paepe MD, PhD, Y Benoit MD, PhD, F. Speleman PhD, P. Van Vlierberghe PhD, B. De Moerloose MD, PhD

Summary

Juvenile myelomonocytic leukaemia is a very rare, aggressive stem cell disorder predominantly affecting infants and young children. Current survival rates are disappointing and the only available curative therapy is haematopoietic stem cell transplantation. Over the last years, intensive research efforts elucidated a plethora of molecular aberrations involved in the pathogenesis of juvenile myelomonocytic leukaemia. Current investigations are mainly directed towards the complete unravelling of the molecular biology behind the disease in order to find more specific drugs. This review will focus on the diagnosis, genomic characterisation and the use of experimental therapies in juvenile myelomonocytic leukaemia.

(BELG J HEMATOL 2014; 5(4): 119–24)

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