A. Janssens MD, PhD

INTRODUCTION

BALT lymphomas: A retrospective analysis at the University Hospitals Leuven

SUMMARY

Bronchus-associated lymphoid tissue (BALT) lymphoma is a rare indolent non-Hodgkin lymphoma (NHL). It is a primary pulmonary marginal zone lymphoma originating from the BALT. Diagnosis is often delayed due to nonspecific symptoms, wide variability and low specificity of radiological patterns, and difficulties in getting representative biopsies.¹ There are several treatment options: active surveillance, local therapy (surgery or radiotherapy), or systemic therapy ((chemo)immunotherapy, immunomodulating, or targeted therapy). ^1–3 In this retrospective analysis, 30 patients diagnosed with primary BALT lymphoma were enrolled. The median time to diagnosis was six months (IQR 1.5 – 15.5 months). Median follow-up time was 67.3 months (IQR 30.6 – 106.1). Our review shows that the watch-and-wait approach is a valid option for a newly diagnosed BALT lymphoma, as we see no negative impact on outcome in our small case series. There is no evidence that postponing the start of systemic therapy holds a risk for transformation, which would mean an inferior outcome. Treatment must be guided primarily by symptomatology and not only by radiologic alterations.

(BELG J HEMATOL 2024;15(7):286–97)

Zanubrutinib, a next-generation Bruton’s tyrosine kinase inhibitor

SUMMARY

Bruton’s tyrosine kinase inhibitors (BTKis) have demonstrated impressive clinical activity and tolerability in several B-cell malignancies, both as single agents and in combination with anti-CD20 monoclonal antibodies. The reimbursement for zanubrutinib, a next-generation BTKi, has recently been extended by the Belgian national public health insurance from Waldenström macroglobulinaemia (WM) to chronic lymphocytic leukaemia (CLL) and marginal zone lymphoma (MZL). This review describes the mechanism of action, dosage and administration, efficacy, and tolerability of zanubrutinib.

(BELG J HEMATOL 2024;15(7):269–80)

INTRODUCTION

Understanding hairy cell leukaemia after analysing the University Hospitals Leuven database retrospectively

SUMMARY

Hairy cell leukaemia (HCL) is a rare hematologic malignancy with high response rates after purine-analogue based therapy and an excellent long-term prognosis. We reviewed 104 HCL patients diagnosed between 1980 and 2022 at the University Hospitals Leuven to analyse long-term outcomes and complications. Median follow-up was twelve years. In total, 96 patients (92%) received a first-line treatment consisting of splenectomy (n=13), interferon-α (n=13) and cladribine (n=70). This last therapy resulted in the best response rates (overall response (OR) 99%, complete response (CR) 71%) and a long-lasting progression-free-survival (median PFS ten years). Forty-three percent of patients received multiple therapies for subsequent relapses. The median number of treatment lines was one. The median overall survival (OS) was 30.8 years with a 5-year and 10-year OS of approximately 98% and 91%. Although the prognosis of HCL patients is very good, infections and second malignancies are frequently observed. In this cohort, 55% of the patients had a major infection with an infection-related mortality of 3%. After diagnosis, 22% of the HCL patients developed one or more second malignancies, ranging from 1–3 per patient, with a 10-year cumulative risk of 14.5%.

(BELG J HEMATOL 2024;15(6):249–56)

INTRODUCTION

Highlights in chronic lymphocytic leukaemia

SUMMARY

At EHA 2024 new information on TP53 mutations in chronic lymphocytic leukemia (CLL) were presented together with preliminary or more mature data on doublets and triplets of novel agents. In the session on novel therapies in relapsed/refractory (R/R) CLL we were informed on the preliminary efficacy and safety of two BTK degraders (BGB-16673,NX-5948) and on the results of the bcl-2 inhibitor sonrotoclax in combination with zanubrutinib. Preliminary results on characteristics associated with response to lisocabtagene maraleucel (Liso-cel) in R/R CLL were also communicated.

(BELG J HEMATOL 2024;15(5):184–89)