Charalampia Kyriakou

• MD, PhD
• Consultant Haematologist at University College London

Introduction Belantamab mafodotin (belamaf) was the first BCMA‐targeting immunotherapy licensed in myeloma and was available as monotherapy for a fifth or greater line of treatment. Outcomes for patients in the United Kingdom and the Republic of Ireland potentially differ from those of other regions and may illuminate factors predicting response to...

Background There is emerging evidence for the role of exercise in optimising function, quality of life (QoL) and reducing hospital length-of-stay if commenced prior to undergoing autologous stem cell transplantation (ASCT). A local pilot study of a prehabilitation and rehabilitation intervention during ASCT for myeloma patients indicated promising...

Background Central nervous system involvement with Waldenstrom's Macroglobulinaemia (WM) or lymphoplasmacytic lymphoma (LPL) is termed Bing-Neel syndrome (BNS). An infrequent complication, it nevertheless causes significant morbidity. Optimum treatment sequencing remains to be established, with traditional CNS-penetrating chemotherapy agents and no...

Marginal zone lymphomas (MZL) are a heterogenous group of indolent B-cell non-Hodgkin lymphoma, usually characterized by a non-aggressive course with a long survival for the majority of the patients. When they are symptomatic, patients usually respond to immuno-chemotherapy. However, a small group of patients relapse early or are refractory to stan...

Background Multiple myeloma (MM) patients often develop bone disease, predisposing them to skeletal-related events (SREs). Renal impairment (RI), a cardinal feature of MM, further complicates treatment strategies. Denosumab, a bone-directed immunotherapy, has demonstrated non-inferiority to zoledronic acid but its efficacy and safety in patients wi...

Background: Body composition (BC) refers to the relative proportions of muscle, bone, subcutaneous and visceral adipose tissue for an individual. The association between obesity and a range of health conditions including cancer is well recognised but the Body Mass Index (BMI) commonly used in clinical assessments does not take into consideration th...

Background Waldenstrom's macroglobulinaemia (WM) and non-IgM lymphoplasmacytic lymphoma (LPL) are low grade lymphoproliferative disorders and distinct clinicopathological entities. Although a predominantly bone marrow-based disease with varying degree of plasmacytic differentiation, unlike multiple myeloma focal bone involvement or bony sequalae ar...

Waldenström's Macroglobulinemia (WM) is a rare low-grade B- cell non -Hodgkin lymphoma. Novel therapies have achieved prolonged progression-free (PFS) and overall (OS) survivals. The future role of haemopoietic stem cell transplantation (HSCT), autologous (AHSCT) and allogeneic (allo-HSCT) in the era of the new therapeutic advances needs a critical...

Introduction: Waldenström Macroglobulinaemia (WM) is a low-grade B-cell lymphoma characterised by lymphoplasmacytic marrow infiltration, historically described in white cohorts. The International Prognostic Scoring System for Waldenstrom Macroglobulinemia(IPSSWM) is a staging system used to stratify risk of symptomatic patients requiring treatment...

Introduction: WhiMSICAL (Waldenström Macroglobulinemia Study Involving CArt-wheeL) is a global Waldenström Macroglobulinemia (WM) patient-derived data registry that aims to address important research questions in treatment of WM, linked with longitudinal quality of life (QoL) data. Bendamustine and Rituximab (BR) is often the standard first-line tr...

Introduction: Spinal involvement, a cardinal manifestation of multiple myeloma (MM) bone disease has been identified as a significant prognostic factor for inferior survival. A subgroup of patients with spinal disease and vertebral fractures were traditionally managed with either minor or major surgical intervention for spinal stability. The litera...

Follicular lymphoma (FL) is characterized by repeated relapses. Chemo-immunotherapy leads to durable response in most patients in need for treatment. However, a subset of patients presenting a more aggressive disease course or early relapses can be offered hematopoietic stem cell transplantation (HSCT) as a potentially curative option. The role of...

not included as this is letter to the editor.

High‐dose melphalan followed by stem cell rescue is the standard consolidative therapy for transplant‐eligible patients with multiple myeloma (MM) in the United Kingdom. A melphalan dose of 200 mg/m ² (Mel200) is considered the “gold standard” for autologous stem cell transplant (ASCT) conditioning for fit patients ≤70 years old; however, with a pe...

Purpose Continuous lenalidomide maintenance treatment after autologous stem cell transplantation delivers improvement in progression free and overall survival among newly diagnosed multiple myeloma patients and has been the standard of care in the UK since March 2021. However, there is scant information about its impact on patients’ day-to-day live...

Introduction Autologous haematopoietic stem cell transplantation (aHSCT) is increasingly used as treatment for patients with active multiple sclerosis (MS), typically after failure of disease-modifying therapies (DMTs). A recent phase III trial, ‘Multiple Sclerosis International Stem Cell Transplant, MIST’, showed that aHSCT resulted in prolonged t...

Objective To describe determinants of persisting humoral and cellular immune response to the second COVID‐19 vaccination among patients with myeloma. Methods This is a prospective, observational study utilising the RUDYstudy.org platform. Participants reported their second and third COVID‐19 vaccination dates. Myeloma patients had an Anti‐S antibo...

Introduction: BCMA targeted therapies have demonstrated single agent efficacy for patients with triple class exposed or refractory multiple myeloma (RRMM). Teclistamab and Belantamab Mafodotin (Belamaf) are currently approved for RRMM in Europe with several Bispecific T Cell Engagers (TCE) under investigation. However, whilst TCEs have higher respo...

Background: Randomised trials examining continuous lenalidomide maintenance after autologous stem cell transplantation (ASCT) in patients with multiple myeloma (MM) have demonstrated progression free survival (PFS) and overall survival benefit compared to placebo. In 2021 the UK National Institute of Clinical Excellence (NICE) approved lenalidomide...

Introduction: Waldenström Macroglobulinaemia (WM) is a low-grade B-cell lymphoma characterised by lymphoplasmacytic marrow infiltration with a circulating IgM protein with an incidence of approximately 0.55 cases per 100,000 in the United Kingdom (UK). WM may be asymptomatic, symptomatic and/or associated with IgM-related entities (monoclonal gammo...

Purpose Continuous lenalidomide maintenance treatment after autologous stem cell transplantation delivers improvement in progression free and overall survival among newly diagnosed multiple myeloma patients and has been the standard of care in the UK since March 2021. However, there is scant information about its impact on patients’ day-to-day live...

Hodgkin lymphoma (HL) has become one of the most curable cancers. Therefore, rigorous assessment of health-related quality of life (HRQoL) and symptom burden of these patients is essential to support informed clinical decisions. This paper reports the final results of an international study by the European Organisation for Research and Treatment of...

Monoclonal immunoglobulin M‐associated type I cryoglobulinaemia is poorly characterised. We screened 534 patients with monoclonal IgM disorders over a 9‐year period and identified 134 patients with IgM type I cryoglobulins. Of these, 76% had Waldenström macroglobulinaemia (WM), 5% had other non‐Hodgkin lymphoma (NHL) and 19% had IgM monoclonal gamm...

Background: Bing-Neel syndrome (BNS) is a complication of lymphoplasmacytic lymphoma (LPL) comprising LPL infiltration in the central nervous system (CNS). There is no standard of care; both chemoimmunotherapy (CIT) and BTK inhibitors are effective. The use of intensive CNS-penetrating CIT is well described in aggressive lymphomas, but there are li...

Background Bing-Neel syndrome (BNS) is a complication of lymphoplasmacytic lymphoma (LPL) comprising LPL infiltration in the central nervous system (CNS). There is no standard of care; both chemoimmunotherapy (CIT) and BTK inhibitors are effective. The use of intensive CNS-penetrating CIT is well described in aggressive lymphomas, but there are l...

High dose melphalan (HDM) followed by autologous stem cell transplantation (ASCT) remains the standard consolidation in transplant eligible multiple myeloma (MM) patients. The timing between HDM administration and hematopoietic stem cell return (HSCR) varies among institutions, with a 'rest period' of 48 hours (h) employed by some for patients with...

Background Health‐related quality of life (HRQOL) is a critical aspect to consider when making treatment decisions for patients with non‐Hodgkin‐lymphoma (NHL). This international study by the European Organisation for Research and Treatment of Cancer (EORTC) tested the psychometric properties of two newly developed measures for patients with high‐...

The European Organisation for Research and Treatment of Cancer Quality of Life Multiple Myeloma Questionnaire (EORTC QLQ-MY20) was developed in 1996 to assess health-related quality of life (HRQoL) in patients with multiple myeloma. Since its development new therapies have prolonged survival in patients with myeloma and new combination agents are l...

Systemic AL amyloidosis is an incurable condition with various presentations and may cause multiple complications related to organ involvement. As survival has improved, disease and therapy‐related quality of life (QoL) is becoming an increasingly important treatment endpoint. We review the literature summarising the utilised QoL questionnaires (QL...

Allogeneic transplantation (allo-HCT) is a curative treatment in CLL whose efficacy including the most severe forms had led to the 2006 EBMT recommendations. The advent after 2014 of targeted therapies has revolutionized CLL management, allowing prolonged control to patients who have failed immunochemotherapy and/or have TP53 alterations. We analys...

Bendamustine and rituximab (BR) therapy is commonly used in the treatment of Waldenström Macroglobulinemia (WM). The impact dose of Bendamustine dose on response and survival outcomes is not well‐established, and the impact of its use in different treatment settings is not clear. We aimed to report response rates and survival outcomes following BR,...

National registries are used globally to characterise patient demographics, treatment choices and mortality to inform and improve clinical management. Waldenström macroglobulinaemia (WM) is a rare, treatment‐responsive B‐cell lymphoproliferative disorder with diverse clinical features and variable outcomes. To prospectively chart changes in the man...

Waldenström macroglobulinaemia (WM) is an incurable chronic B‐cell malignancy, but highly responsive to treatment. Treatments include fixed‐duration chemotherapy and continuous oral chemoimmunotherapy. In this expanding field, it is important to have reliable information on the impact of the various therapies on patients’ quality of life (QoL). Pat...

The diagnosis of Waldenström’s macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. Furthermore, in recent years, WM has witnessed remarkable progress on the diagnostic front, as well as a deeper understanding of the disease biology, which has affected clinical...

Waldenström macroglobulinemia (WM) is a rare, incurable low grade lymphoma following a relapsing trajectory. Management strategies have evolved with the introduction of targeted therapy including new classes of Bruton tyrosine kinase inhibitor (BTKi). Treatment may however be limited particularly at relapse by a lack of drug availability and tolera...

The diagnosis of Waldenström’s macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, can be challenging due to the different forms of disease presentation. Furthermore, in recent years, WM has witnessed remarkable progress on the diagnostic front, as well as a deeper understanding of the disease biology, which has affected clinical...

Autologous stem cell transplantation (ASCT) is standard of care in biologically fit, newly diagnosed multiple myeloma (MM) patients, offering better therapeutic outcomes and improved quality of life (QoL). However, with the UK’s 1st national lockdown on 23/03/2020, several guidelines recommended deferring ASCT due to risks of infection, with resour...

Background The clinical management of patients with relapsed or refractory multiple myeloma is challenging and there is a paucity of tools to help clinicians make more informed decisions for the most suitable treatment options. We aimed to investigate the clinical utility of the International Myeloma Working Group (IMWG) frailty score in the settin...

Depth of response is the critical determinant of prognosis in amyloid light‐chain (AL) amyloidosis. Here, we aim to identify patients who are unlikely to improve response based on analysis of baseline characteristics and 1‐month response. In a multivariate model, difference in involved amyloidogenic and uninvolved serum free light chains (dFLC) at...

Background: Waldenstrom’s Macroglobulinaemia (WM) is a rare disorder that is associated with a broad spectrum of symptoms. The severity of the presentation varies from asymptomatic to symptomatic, high-grade transformation, central nervous system disease and development of autoimmune phenomena. Thus, the treatment approach for WM is heterogeneous...

Autologous stem cell transplantation (ASCT) is standard of care in biologically fit, newly diagnosed multiple myeloma (MM) patients, offering better therapeutic outcomes and improved quality of life (QoL). However, with the UK’s 1st national lockdown on 23/03/2020, several guidelines recommended deferring ASCT due to risks of infection, with resour...

Selecting the most appropriate chronic lymphocytic leukaemia (CLL) treatment is challenging. Patient‐reported health‐related quality of life (HRQoL) is therefore a critical aspect to consider. This international study by the European Organization for Research and Treatment of Cancer (EORTC) tested the psychometric properties of a newly developed me...

Purpose: High mortality and a limited performance of valvular surgery are typical features of infective endocarditis (IE) in octogenarians, even though surgical treatment is a major determinant of a successful outcome in IE. Methods: Data from the prospective multicentre ESC EORP EURO-ENDO registry were used to assess the prognostic role of valv...

Myeloma patients frequently respond poorly to bacterial and viral vaccination. A few studies have reported poor humoral immune responses in myeloma patients to COVID‐19 vaccination. Using a prospective study of myeloma patients in the UK Rudy study cohort, we assessed humoral and interferon gamma release assay (IGRA) cellular immune responses to CO...

Background: Consolidative autologous stem cell transplantation (ASCT), incorporating high dose melphalan (HDM) for conditioning, is the UK standard of care for transplant-eligible multiple myeloma (MM) patients. Melphalan undergoes rapid hydrolysis in plasma with a short half-life (60-90 minutes). The duration between HDM and haematopoietic stem ce...

Scope The objective of this guideline is to provide healthcare professionals with clear guidance on the management of patients with Waldenström macroglobulinaemia. In individual patients, circumstances may dictate an alternative approach. Methodology This guideline was compiled according to the British Society for Haematology (BSH) process at http...

BACKGROUND Ibrutinib is approved as a second line treatment option for Waldenström Macroglobulinemia (WM) patients in the United Kingdom and for patients unsuitable for chemo-immunotherapy. In a clinical trial setting, Ibrutinib monotherapy produced a major response rate (MRR) of 79.4% and a predicted five-year overall survival (OS) rate of 87%1. V...

Background: Treatment of multiple myeloma (MM) commonly encompasses combination of drugs within the three drug classes including proteasome inhibitors (PIs), immunomodulators and (IMiDs), and monoclonal antibodies, both for newly diagnosed and relapsed disease. With increasing appreciation of disease heterogeneity based on underlying genetic abnorm...

Background: Systemic AL Amyloidosis is characterized by deposition by amyloid fibrils of light chains produced by clonal plasma cells. New combination therapies have substantially prolonged the life expectancy of patients with AL Amyloidosis . Still the prognosis of the disease in the majority of the patients is dismal and Quality of Life (QoL) iss...

Introduction: Waldenström's Macroglobulinaemia (WM) is a rare indolent B-cell lymphoma characterised by lymphoplasmacytic infiltrate with an IgM paraproteinaemia. Approximately 400 patients (pts) are diagnosed annually in the United Kingdom (UK). National and regional registries provide important insights on the epidemiology and clinical features o...

Background Plasma cell disorders (PCD) are at risk of inadequate immune responses to COVID-19 vaccines due to recognised humoral and cellular immune dysfunction which is multi-factorial and related to host and disease factors. With an estimated risk of 33% mortality from contracting COVID-19 in this population, protection with an anti-SARS-CoV-2 va...

Introduction Type 1 cryoglobulinaemia (CG) is characterised by monoclonal immunoglobulins which precipitate at temperatures below 37°C and redissolve on warming. They may be associated with lymphoproliferative disorders including Waldenström's macroglobulinemia (WM), other non-Hodgkin lymphoma (NHL), chronic lymphocytic leukaemia (CLL) or monoclona...

Background Clinical decision-making in patients with relapsed refractory multiple myeloma (RRMM) is challenging and identification of patients who can benefit the most from a given therapy is of critical importance. The International Myeloma Working Group (IMWG) index is one of the most robust scores to evaluate frailty, and it has been validated f...