• Issue

    British Journal of Haematology: Volume 206, Issue 3

    805-1021
    March 2025

Issue Information

Free Access

Issue Information

  • Pages: 805-808
  • First Published: 07 March 2025

REVIEW

Free to Read

Update on B-cell maturation antigen-directed therapies in AL amyloidosis

  • Pages: 817-831
  • First Published: 02 January 2025
Update on B-cell maturation antigen-directed therapies in AL amyloidosis

Despite the significant progress in the treatment of systemic light chain (AL) amyloidosis with the introduction of the anti-CD38 monoclonal antibody daratumumab to the first-line therapy, relapsed or refractory disease remains an unmet medical need. B-cell maturation antigen (BCMA) is overexpressed on the surface of clonal plasma cells in AL amyloidosis and, therefore, represents a potential target for immunotherapy. Recent results from small clinical trials and retrospective studies suggest high efficacy of anti-BCMA strategies in relapsed refractory AL amyloidosis, including therapies already approved for multiple myeloma (antibody–drug conjugates belantamab mafodotin, bispecific T-cell redirecting antibodies teclistamab and elranatamab and chimeric T-cell antigen receptor therapies idecabtagene vicleucel and ciltacabtagene autoleucel) as well as novel immunotherapies in early clinical development.

Open Access

Management strategies for patients with chronic lymphocytic leukaemia harbouring complex karyotype

  • Pages: 832-841
  • First Published: 06 January 2025
Management strategies for patients with chronic lymphocytic leukaemia harbouring complex karyotype

Chronic Lymphocytic Leukaemia patients harbouring complex karyotype presents poor prognosis and limited treatment options due to its intrinsic genomic instability. This review aims to discuss the current understanding of such patient subset, including its molecular landscape, diagnostic approaches, treatment modalities and emerging therapies. Furthermore, it outlines strategies for personalised management to improve clinical outcomes in this challenging patient population.

WIDER PERSPECTIVES

Open Access

Sickle cell anaemia therapy in 2025

  • Pages: 842-845
  • First Published: 22 December 2024

ORIGINAL PAPER

Haematological Malignancy – Clinical

Open Access

Acalabrutinib in combination with rituximab and lenalidomide in patients with relapsed or refractory follicular lymphoma: Results of the phase 1b open-label study (ACE-LY-003)

  • Pages: 887-898
  • First Published: 12 December 2024
Acalabrutinib in combination with rituximab and lenalidomide in patients with relapsed or refractory follicular lymphoma: Results of the phase 1b open-label study (ACE-LY-003)

This phase 1b open-label, dose-finding study evaluated acalabrutinib in combination with rituximab plus lenalidomide 15 or 20 mg in 29 patients with relapsed or refractory follicular lymphoma. The combination treatment had acceptable toxicity with promising clinical activity. At a median acalabrutinib exposure of 21 months, the most common grade ≥3 treatment-emergent adverse event was neutropenia, occurring in 11 (38%) patients, with no cases of febrile neutropenia. Among all treated patients, the overall response rate was 76% at a median follow-up of 34.1 months. A numerically higher complete response rate was seen in patients in the lenalidomide 20-mg group (43%) versus the 15-mg group (25%). Results from this study led to the selection of the 20-mg lenalidomide dose for further study.

Paediatrics

Should adolescents and young adults with Hodgkin lymphoma be treated as children or adults?

  • Pages: 907-918
  • First Published: 05 January 2025
Should adolescents and young adults with Hodgkin lymphoma be treated as children or adults?

Hodgkin lymphoma (HL) is one of the most common cancers in adolescents and young adults (AYA) yet no treatment specifically tailored to this age group currently exists. This study compared 148 patients aged 15 to 25 treated at Saint-Louis Hospital between 2012 and 2018, using either pediatric or adult protocols. A higher risk of short-term steroid and vincristine-related toxicities was observed in pediatric regimen, whereas a higher risk of late toxicities was expected in adult regimen, due to higher anthracyclines, procarbazine, bleomycin and radiotherapy exposure. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 100% and 85%, respectively, with no significant difference between the two approaches. These findings emphasize the importance of personalized care in specialized units to optimize treatment outcomes while minimizing short- and long-term toxicities.

ORIGINAL PAPER

Haemoglobinopathies

Depression, sleep and pain affect instrumental activities of daily living through cognitive functioning in adults with sickle cell disease: A report from the Sickle Cell Disease Implementation Consortium

  • Pages: 944-953
  • First Published: 20 November 2024
Depression, sleep and pain affect instrumental activities of daily living through cognitive functioning in adults with sickle cell disease: A report from the Sickle Cell Disease Implementation Consortium

Depression, disrupted sleep and pain are common comorbidities in sickle cell disease. These comorbidities were associated with patient-reported difficulties in processing speed and attention/executive functioning when controlling for stroke and demographics. The indirect effects of attention/executive functioning and processing speed were significant in mediation models that examined pathways between disease-related comorbidities and instrumental activities of daily living (IADLs), which represent complex skills required to support independence. Difficulties with attention/executive functioning accounted for nearly 20% of the relationship between depression and IADLs and sleep and IADLs. Managing comorbidities in adults may assist in minimizing cognitive symptoms and improving IADLs.

Open Access

Plasma inflammatory and angiogenic protein profiling of patients with sickle cell disease

  • Pages: 954-964
  • First Published: 01 January 2025
Plasma inflammatory and angiogenic protein profiling of patients with sickle cell disease

The results of these proteomic analyses reveal 50 proteins involved in inflammation and angiogenesis that are differentially expressed in patients with sickle cell disease in steady state as compared to healthy ethnicity-matched controls. These included proteins involved in angiogenesis (i.e. ANGPT1, ANGPT2 and VEGFA), the IL-18 signalling pathway (i.e. IL-6, IL-10, IL-18), T-cell activation (i.e. LAG3, PDCD1) and NK-cell activation (CD244, NCR1, GZMB). While proteins involved in angiogenesis and the IL-18 signalling pathway were further upregulated during a vaso-occlusive episode, levels of several proteins involved in the IL-18 pathway, T-cell and NK-cell activation and angiogenesis, restored towards levels detected in HCs after curative or disease-modifying treatment.

Transfusion

Open Access

Understanding variations in the use of tranexamic acid in surgery: A qualitative interview study

  • Pages: 965-976
  • First Published: 18 February 2025
Understanding variations in the use of tranexamic acid in surgery: A qualitative interview study

The use of tranexamic acid (TXA) to reduce surgical bleeding is highly variable with scope for improvement. Effective implementation strategies should be based on an understanding of clinical behaviour. We interviewed a range of clinicians involved in perioperative care to elicit key influences on TXA use. These included the clinical context, variable familiarity with TXA, the availability of both TXA and checklists to support decision-making, professional expectations and perceived responsibilities, confidence in administering TXA and perceived benefits and risks. These influences, which varied across participants and specialities, offer targets for coordinated implementation strategy.

LETTER TO THE EDITOR

COMMENTARY