Articles

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

BJH - volume 15, issue 4, june 2022

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)

Read more

Straight to the heart

BJH - volume 14, issue 7, november 2023

H. Lismont MD, T. Tousseyn MD, PhD, D. Dierickx MD, PhD

SUMMARY

We report the case of a 56-year-old patient with medical history of acute myeloid leukaemia (AML), presenting with shortness of breath and lower extremity oedema. Transthoracic echocardiogram revealed an important amount of pericardial fluid and an infiltrating mass located at the left ventricular wall. A pericardial window with drainage was performed and a biopsy of the pericardium was taken. The pathological report was compatible with an extramedullary manifestation of AML. Further work-up with complete blood test and bone marrow biopsy confirmed a systemic AML relapse. The patient was treated with re-induction chemotherapy and cardiac radiotherapy followed by a second allogeneic stem cell transplantation, leading to a complete remission.

(BELG J HEMATOL 2023;14(7):304–7)

Read more

BHS guidelines on the management of relapsed and refractory diffuse large B-cell lymphoma: Part 2

BJH - volume 14, issue 4, june 2023

U. Douven MD, A. Janssens MD, PhD, G. Crochet MD, S. Bailly MD, C. Bonnet MD, C. Jacquy MD, PhD, F. Offner MD, PhD, S. Snauwaert MD, PhD, E. Van den Neste MD, PhD, M. Vercruyssen MD, D. Dierickx MD, PhD, P. Vandenberghe MD, PhD, V. Vergote MD

SUMMARY

Approximately 30–40% of patients with diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS), will relapse or are unable to obtain a complete remission (CR) after frontline treatment. These patients have a poor prognosis and represent a therapeutic challenge. In this article, we reviewed the recent literature to update the practice guidelines of the Belgian Hematology Society (BHS) Lymphoproliferative Disease Committee for the treatment of relapsed or refractory (R/R) DLBCL. In the first part, we will focus on first relapse and the role of CAR T-cell therapy in first and second relapse. In the second part, we will focus on novel treatment options for patients with a second or higher relapse, secondary central nervous system (CNS) relapse and high-grade lymphoma.

(BELG J HEMATOL 2023;14(4):170–7)

Read more

BHS guidelines on the management of relapsed and refractory diffuse large B-cell lymphoma: Part 1

BJH - volume 14, issue 3, may 2023

U. Douven MD, A. Janssens MD, PhD, G. Crochet MD, S. Bailly MD, C. Bonnet MD, C. Jacquy MD, PhD, F. Offner MD, PhD, S. Snauwaert MD, PhD, E. Van den Neste MD, PhD, M. Vercruyssen MD, D. Dierickx MD, PhD, P. Vandenberghe MD, PhD, V. Vergote MD

SUMMARY

Approximately 30–40% of patients with diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS), will relapse or are unable to obtain a complete remission (CR) after frontline treatment. These patients have a poor prognosis and represent a therapeutic challenge. In this article, we reviewed the recent literature to update the practice guidelines of the Belgian Hematology Society (BHS) Lymphoproliferative Disease Committee for the treatment of relapsed or refractory (R/R) DLBCL. In the first part, we will focus on first relapse and the role of CAR T-cell therapy in first and second relapse. In the second part, we will focus on novel treatment options for patients with a second or higher relapse, secondary central nervous system (CNS) relapse and high-grade lymphoma.

(BELG J HEMATOL 2023;14(3):114–21)

Read more

Primary vitreoretinal lymphoma: Single centre experience and review of literature

BJH - volume 13, issue 6, october 2022

C. Debergh MD, A. Janssens MD, PhD, D. Dierickx MD, PhD, R. Van Ginderdeuren MD, T. Tousseyn MD, PhD, J. Van Calster MD, V. Vergote MD

SUMMARY

BACKGROUND: Primary vitreoretinal lymphoma (PVRL) is a rare and difficult to diagnose lymphoma. The goal of this retrospective monocentric study was to obtain clinical characteristics, to evaluate median time to diagnosis, different treatment modalities and survival outcomes.

METHODS: PVRL cases were selected from the database of the University Hospitals Leuven (Belgium) from 1st January 2012 until 1st January 2021. A review of the available literature was performed.

RESULTS: We included eleven cases of PVRL with a median age of 76 years (Interquartile range (IQR): 68–81). Median time to diagnosis was seven months (Range: 3–16). Presenting symptoms were blurred vision (n=11, 100%) and floaters (n=3, 27%). Bilateral eye involvement was seen in 42% (n=5). Diagnosis was made by vitrectomy and immunocytochemistry in all cases. Histopathological diagnosis was diffuse large B-cell lymphoma in all cases. Flow cytometry was used in 55% (n=6) of patients to confirm diagnosis. Initial treatment included local therapy in all patients. A combination of local and systemic therapy was given to three patients (27%). Seven patients (64%) were diagnosed with CNS relapse. No systemic relapse was seen. Median progression-free survival (PFS) and overall survival (OS) were ten (IQR: 6–32) and 26 months (IQR: 12–37). Median PFS of patients treated with local versus combined therapy was 9.7 and 18 months, respectively. However, OS of patients with local versus combination therapy was 29 and 19 months, respectively.

CONCLUSION: We analysed the clinical characteristics of eleven patients with PVRL in our hospital. The majority of these cases will eventually progress to CNS lymphoma. We saw a prolonged PFS for patients treated with combination therapy in first-line, compared to local therapy alone. However, OS was longer in patients treated with local therapy only. Despite the small cohort, these results are comparable to previous literature. Based on larger retrospective studies we conclude that local therapy as first line treatment in PVRL results in similar OS rates with less systemic toxicity compared to combination therapy.

(BELG J HEMATOL 2022;13(6):228–235)

Read more

Immunity, the Epstein-Barr virus and the microenvironment in lymphoma

BJH - volume 12, issue 6, october 2021

L. Marcelis MD, PhD, R. Snoeck MD, PhD, D. Dierickx MD, PhD, T. Tousseyn MD, PhD

SUMMARY

Immune regulation therapy or ‘immunotherapy’ has been a major evolution in the field of cancer therapy in the last decade. The goal of this thesis was to better characterise multiple rare lymphoproliferative disorders in order to guide therapy development, predictive biomarker discovery and ultimately help ensuring that these novel therapies can get to the patients who stand to benefit from them. Many lymphoma types arise in a context of altered immune system function with potential implications for immunotherapy. One example of lymphoma arising in the context of chronic immune stimulation is Helico bacter Pylori infection, which is known as mucosa associated lymphoid tissue (MALT) lymphoma of the stomach. For this lymphoma we reviewed the literature and described how it is an excellent model to understand lymphomas arising in an immune stimulated context.1 Other lymphomas arise in a context of immunosuppression, of which post-transplant lymphoproliferative disorders (PTLD) are the best characterised. Besides the immune state, the presence of a virus such as the Epstein-Barr virus has major effects on lymphomas and their (immune) microenvironment with potential impact on immunotherapy. A review of EBV-related effects on PTLDs was done.² Lymphoproliferative disorder’s (LPDs) arising in the context of treatment with immunomodulatory (IM)/immunosuppressive (IS) drugs for various auto-immune diseases are lesser-known. These are called immunomodulation related lymphoproliferative disorders (IARLPD). For this thesis, we characterised one of the largest single centre case series of IARLPD.³ Finally, some lymphomas arise in specific ‘immune-privileged’ sites such as the central nervous system (CNS) called Primary central nervous system lymphoma (PCSNL). Digital slide analysis method and the novel MILAN multiplex staining technique were used to study the tumour microenvironment (TME) in PCNSL confirming the relevance of the microenvironment in the clinical behaviour of this lymphomas, highlighting potential relevance of immunotherapy and confirming the usefulness of the mentioned techniques in the study of the TME.4 For the purpose of this dissertation presentation we will focus primarily on this latter work.

(BELG J HEMATOL 2021;12(6):280-2)

Read more

EBV-induced double hit transformation of splenic (marginal zone) lymphoma, in a patient with a chronic Hepatitis B virus hepatitis

BJH - volume 12, issue 6, october 2021

L. Jannis MD, L. Waumans MD, L. Michaux MD, PhD, C. Deroose MD, PhD, D. Dierickx MD, PhD, T. Tousseyn MD, PhD

SUMMARY

We report a case of an EBV-induced large-cell transformation of a splenic marginal zone lymphoma in an 81-year-old female with a simultaneous finding of hepatitis B viral infection.

(BELG J HEMATOL 2021;12(6):275-9)

Read more