Long-term outcome following vertebroplasty or bone cementoplasty in multiple myeloma patients.

Objective: Evaluating long-term outcomes following cementoplasty in patients with multiple myeloma (MM).

Methods: This is a single-center, retrospective study on all cementoplasties performed between January 2012 and December 2017. Patients with MM with a control MRI or CT scan beyond 5 years after the procedure were included.

Real-world efficacy and safety outcomes of acalabrutinib in chronic lymphocytic leukemia: primary results of a French multicentre observational study (NAOS).

Acalabrutinib is a second-generation BTK inhibitor approved for the treatment of chronic lymphocytic leukemia (CLL) after demonstrating efficacy and safety in clinical trials. NAOS, a real-world study, complements these trials with real-world (rw) acalabrutinib data generated from clinical practice. This retrospective, non-interventional, longitudinal study was conducted in 59 sites in France in adult patients initiating acalabrutinib for CLL in 2021-2022.

Report of Consensus Panel 2 from the 12th International Workshop on the management of Bing-Neel syndrome in patients with Waldenstrom's Macroglobulinemia.

Consensus panel 2 from the 12th International Workshop on Waldenstrom Macroglobulinemia was tasked with updating the guidelines on the diagnosis and management of patients with Bing-Neel syndrome (BNS). In this panel we have summarized the clinical symptoms that may be present with BNS, discussed the criteria required for diagnosis of BNS, made recommendations for follow-up imaging, and proposed revised guidelines for response assessment in BNS. The key recommendations from the 12th International Workshop on WM (IWWM-12) Consensus panel 2 include: (1) the establishment of zanubrutinib as a standard therapy for treatment of BNS; (2) recommendations on imaging and CSF evaluation during treatment and follow-up of BNS; and (3) revised response criteria in view of new data showing that malignant cells can persist in the CSF of many patients treated with BTK-inhibitors.

Phenotypic Profile of Waldenström Macroglobulinaemia B-Cells: Establishment of a Diagnosis Scoring System and Clinico-Biological Correlations.

Waldenström Macroglobulinaemia (WM) is sometimes difficult to differentiate from marginal zone lymphoma (MZL), two entities with overlapping features for which no single marker assessed by multiparameter flow cytometry (MFC) is specific. The aim of this work was to establish a diagnostic phenotypic score for bone marrow (BM) and peripheral blood (PB) WM samples, to differentiate it from MZL and to improve the detection of small circulating WM clones. This study revealed a distinct phenotypic profile between WM and MZL B-cells.

Report of Consensus Panel 6 from the 12th International Workshop on Waldenstrom's Macroglobulinemia on Diagnosis and Management of Transformed Waldenstrom's Macroglobulinemia.

Histological transformation (HT) in Waldenström's macroglobulinemia (WM) is a rare complication and despite growing literature in the last years, no consensus recommendations exist. Consensus Panel 6 (CP6) of the 12th International Workshop on Waldenström's Macroglobulinemia (IWWM-12) was convened to review the current data on transformed WM and make recommendations on its diagnosis and management. The key recommendations from IWWM-12 CP6 included: (1) in case of suspected HT, tissue biopsy is the gold standard for diagnosis; (2) the initial work-up should comprise FDG-PET/CT for the evaluation of disease extent and, for patients with clinical suspicion or for high-risk patients (CNS-IPI, multiple and/or specific extranodal involvements), cerebrospinal fluid examination and brain MRI; (3) standard dose chemoimmunotherapy (CIT) such as R-CHOP (rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone) or R-CHP + polatuzumab vedotin are the preferred front-line regimen; (4) CNS prophylaxis and consolidation with autologous stem cell transplantation (SCT) can be considered according to de novo diffuse large B-cell lymphoma (DLBCL) guidelines; (5) T-cell-engaging therapies (CAR T-cells, bispecific antibodies) should be used in the relapse/refractory setting according to international guidelines for DLBCL and local access to these therapies.

A multiomic analysis of Waldenström macroglobulinemia defines distinct disease subtypes.

We carried out a single-cell multiomic analysis on a series of MYD88-mutated Waldenström macroglobulinemia (WM) patients and identified 2 distinct subtypes of disease, memory B-cell (MBC)-like and plasma cell (PC)-like, based on their expression of key lineage defining genes. Biologically, the subtypes are characterized by their variable capacity to differentiate fully toward a PC and exhibit unique transcriptomic, chromatin accessibility, and genomic profiles. The MBC-like subtype is unable to differentiate beyond the MBC stage, upregulates key MBC genes, and is characterized by upregulated B-cell receptor and phosphatidylinositol 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/AKT/mTOR) signaling.

Reduced venetoclax exposure to 7 days vs standard exposure with hypomethylating agents in newly diagnosed AML patients.

Hypomethylating agent (HMA) plus venetoclax (VEN) regimens are standard of care in patients with acute myeloid leukemia (AML) ineligible for intensive chemotherapy. While the VEN label recommends continuous 28-day cycles, shortened VEN durations may induce similar response rates and improve tolerability. It is unknown how a VEN exposure reduced to 7 days during cycles compares to standard HMA + VEN.

Neurotoxicity in Patients With CNS Lymphomas Treated With CAR T-Cell Therapy: A Study From the French Oculo-Cerebral Lymphoma Network.

Background And Objectives: Several recent studies have shown the promising efficacy of chimeric antigenic receptor (CAR) T cells in treating CNS lymphomas. However, data on neurotoxicity in this setting are limited. The objective of this study was to describe neurotoxicity in patients with CNS lymphoma treated with anti-CD19 CAR T cells and to identify risk factors.

Monocytic meningitis complicating histiocytosis and response to MEK-inhibitor: a case series.

Central nervous system (CNS) involvement is common in histiocytosis, yet cerebrospinal fluid (CSF) analysis often yields normal results. We present three cases of monocytic meningitis associated with histiocytosis. The first patient was diagnosed with Erdheim-Chester disease (ECD) and exhibited evidence of a MAP2K1 mutation, concomitant with chronic myelomonocytic leukemia.

Bortezomib, Rituximab and Dexamethasone Regimen (BDR) in Waldenström Macroglobulinaemia: A Retrospective Real-World Analysis.

Introduction: We retrospectively analysed bortezomib-dexamethasone-rituximab (BDR) combination in patients with Waldenström macroglobulinaemia (WM) in a real world setting.

Methods: A total of 87 patients were included: 49 patients (56%) were treated in frontline, 22 (25%) in second line and 16 (19%) in third or further line settings. A log-rank test was used to compare overall and event-free survival (OS and EFS) whereas a Gray's test was performed to compare cumulative incidence of deaths and relapse (CID and CIR) according to the IPSS-WM groups, MYD88/CXCR4 mutational status and line of therapy.

Characterization and treatment outcomes of malignant histiocytoses in a retrospective series of 141 cases in France.

Malignant histiocytoses (MH) are rare and poorly understood cancers, with no established therapeutic guidelines. We conducted a national retrospective study of MH diagnosed in France between 2000 and 2023. All cases underwent centralized histological review, and several malignant tumors with a stroma highly enriched in histiocytes were excluded.

Inflammatory Waldenström macroglobulinemia is associated with clonal hematopoiesis: a multicentric cohort.

Inflammatory form of Waldenström macroglobulinemia (iWM) predicts outcomes after immuno-chemotherapy and Bruton tyrosine kinase inhibitors, but its origin is unknown. Here, we unravel increased clonal hematopoiesis in patients with iWM (61% vs 23% in noninflammatory WM), suggesting a contribution of environmental cells to iWM.

Six-year follow-up of phase II study exploring chemo-free treatment association with idelalisib and obinutuzumab in symptomatic relapsed/ refractory patients with Waldenström's macroglobulinemia.

We present the 6-year update of a phase 2 study evaluating the combination of obinutuzumab and idelalisib in relapse/refractory Waldenstrom macroglobulinemia. The results of the REMODEL trial demonstrated interesting efficacy in a high-risk genotype profile population. The primary endpoint was achieved with a median PFS of 25.

Epigenetic features support the diagnosis of B-cell prolymphocytic leukemia and identify 2 clinicobiological subtypes.

The recognition of B-cell prolymphocytic leukemia (B-PLL) as a separate entity is controversial based on the current classification systems. Here, we analyzed the DNA methylome of a cohort of 20 B-PLL cases diagnosed according to the guidelines of the International Consensus Classification/Fourth revised edition of the World Health Organization Classification, and compared them with chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL), splenic marginal zone lymphoma (SMZL), and normal B-cell subpopulations. Unsupervised principal component analyses suggest that B-PLL is epigenetically distinct from CLL, MCL, and SMZL, which is further supported by robust differential methylation signatures in B-PLL.

Blinatumomab after R-CHOP bridging therapy for patients with Richter transformation: a phase 2 multicentre trial.

Richter transformation (RT) is an aggressive lymphoma occurring in patients with chronic lymphocytic leukaemia. Here we investigated the anti-CD3/anti-CD19 T-cell-engager blinatumomab after R-CHOP (i.e.

Impact of second autologous stem-cell transplantation at relapsed multiple myeloma: A French multicentric real-life study.

A second autologous stem-cell transplantation (ASCT2) is considered for relapsed multiple myeloma (RMM) patients showing prolonged response after a first ASCT. However, given breakthrough treatments like anti-CD38 and immunotherapy, its role remains debated. We conducted a real-life study in 10 French centers (1996-2017) involving 267 RMM patients receiving ASCT2.

Epstein-Barr virus and immune status imprint the immunogenomics of non-Hodgkin lymphomas occurring in immune-suppressed environments.

Non-Hodgkin lymphomas (NHL) commonly occur in immunodeficient patients, both those infected by human immunodeficiency virus (HIV) and those who have been transplanted, and are often driven by Epstein-Barr virus (EBV) with cerebral localization, raising the question of tumor immunogenicity, a critical issue for treatment responses. We investigated the immunogenomics of 68 lymphoproliferative disorders from 51 immunodeficient (34 post-transplant, 17 HIV+) and 17 immunocompetent patients. Overall, 72% were large B-cell lymphoma and 25% were primary central nervous system lymphoma, while 40% were EBV+.