Hematological Oncology最新文献

Hairy cell leukemia (HCL) and HCL-like disorders have to be distinguished because of their different biology and treatment response. Thus, we conducted a retrospective study on patients with HCL and hairy cell leukemia variant (HCLv) to assess diagnostic algorithms and treatment outcomes in a real-world setting. We analyzed 225 HCL and 26 HCLv patients with median follow-up of 67.9 months (HCL) and 20.1 months (HCLv). Median age at diagnosis was 56.2 (HCL) and 69.5 years (HCLv), male predominance was observed in both groups (76.0% vs. 73.1%). Diagnostics was mostly based on morphological evidence of hairy cells in the peripheral blood and bone marrow. At diagnosis, BRAF V600E mutation was detected in 94.7% of examined HCL patients and in no HCLv patient. Front-line treatment was indicated in 205 (91.1%) HCL and 18 (69.2%) HCLv patients. The majority of HCL patients were administered a cladribine-based regimen (91.2%). Overall response rate (ORR) was higher in cladribine-treated patients compared to those given other treatments (97.7% vs. 81.3%), the same applied with achieving Complete remission (CR) (91.2% vs. 62.5%). HCLv treatment was heterogeneous, but cladribine remained the most frequent option (44.4%) with ORR 81.3% and CR rates 43.8%. Second-line treatment was indicated in 52 HCL and 8 HCLv patients, 25.4% and 44.4% of those treated in first-line. In the whole HCL group, median time to next treatment (TTNT) was not reached and 10-year TTNT was estimated at 74.1%. HCLv patients who underwent first-line treatment had a median TTNT of 56 months. The median overall survival (OS) in HCL patients was not reached compared to HCLv with a median OS of 9.5 years. These data confirm an excellent prognosis for HCL patients treated with cladribine-based therapy. On the contrary, HCLv with its aggressive behavior represents a group of patients in whom novel treatment approaches are needed.

Follicular lymphoma (FL) is the most common indolent B-cell non-Hodgkin lymphoma. Circulating lymphoma (CL) cells can be seen at diagnosis in some FL patients, however, previous studies evaluating this have shown mixed results. Therefore, we sought to evaluate the impact of CL at diagnosis on outcomes in patients with newly diagnosed FL using data from a single center. Patients were divided into CL+ and CL− based on immunophenotyping via peripheral blood (PB) flow cytometry. CL was defined as detectable clonally restricted B-cells that matched the actual or expected B-cell immunophenotype of FL. The primary endpoint was progression-free survival (PFS) after first-line treatment and secondary endpoints included overall response rate (ORR), overall survival (OS), diagnosis to treatment interval (DTI), progression of disease within 2 years of diagnosis (POD24), and cumulative incidence of transformation between the two groups. Among the 541 patients with FL, 204 had PB flow cytometry performed at diagnosis, and after excluding patients not meeting the eligibility criteria, 147 cases remained with 24 (16%) CL+ at diagnosis. Patients in the CL+ group were younger (53 vs. 58 years, p = 0.02), had more extranodal involvement (83% vs. 44%, p < 0.01), follicular lymphoma international prognostic index 3–5 (55% vs. 31%, p = 0.01), and a higher proportion received first-line immunochemotherapy (75% vs. 43%, p = 0.01) compared to the CL−group. The median PFS was not significantly different between CL+ (6.27 years, 95% CI = 3.61-NR) and CL− (6.61 years, 95% CI = 5.10–9.82) cohorts regardless of the first-line treatment or level of absolute PB CL cells. There was no significant difference in ORR, median OS, DTI, POD24, and cumulative incidence of transformation between the two groups. In our study, we found that the presence of CL cells at diagnosis in FL in the contemporary era did not impact outcomes and survival.

Retraction: “Liposomal-encapsulated doxorubicin supercharge-containing front-line treatment improves response rates in primary mediastinal large B-cell lymphoma and mediastinal gray zone lymphoma” by Picardi, M., Giordano, C., Pugliese, N., Mascolo, M., Varricchio, S., Troncone, G., Vigliar, E., Bellavicine, C., Lamagna, M., Lisi, D., Vincenzi, A. and Pane, F., Hematol Oncol., 2024; 42: e3247. https://doi.org/10.1002/hon.3247.

The above article from Hematological Oncology, published online on 24 January 2024 in Wiley Online Library (wileyonlinelibrary.com), has been retracted by agreement between the journal's editor-in-chief, Francesco Bertoni, the authors, and John Wiley & Sons Ltd. This action has been agreed due to an error at the publishers which caused this duplicate of another article to be published. The correct version of the article, first published online on 21 December 2023, is to be found at: “Liposomal-encapsulated doxorubicin supercharge-containing front-line treatment improves response rates in primary mediastinal large B-cell lymphoma and mediastinal gray zone lymphoma” by Picardi, M., Giordano, C., Pugliese, N., Mascolo, M., Varricchio, S., Troncone, G., Vigliar, E., Bellavicine, C., Lamagna, M., Lisi, D., Vincenzi, A. and Pane, F., Hematol Oncol., 2024; 42: e3242. https://doi.org/10.1002/hon.3242.

Mantle cell lymphoma (MCL) is an uncommon and incurable B-cell lymphoma subtype that has an aggressive course. Hepatitis B virus (HBV) infection has been associated with an increased risk for B-cell lymphomas, and is characterized by distinct clinical and genetic features. Here, we showed that 9.5% of MCL Chinese patients were hepatitis B surface antigen positive (HBsAg⁺). Compared to HBsAg-negative (HBsAg⁻) patients, HBsAg⁺ MCL patients had a greater incidence of elevated lactate dehydrogenase (LDH), but no difference was observed in the other clinical characteristics, including sex, age, ECOG ps, Ann Arbor stage, MIPI, extranodal involvement and Ki-67. The HD-AraC (high-dose cytarabine) regimen was the main first-line induction regimen for younger HBsAg⁺ patients, and cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) were used for elderly patients. HBsAg seropositivity was associated with a significantly shorter PFS than HBsAg seronegativity when patients were treated with rituximab or CHOP-based regimens. Compared with CHOP, the HD-AraC regimen was associated with longer PFS in HBsAg⁺ patients. Treatment with a Bruton tyrosine kinase inhibitor (BTKi) alone can also cause HBV reactivation. Among the 74 patients who underwent targeted deep sequencing (TDS), the nonsynonymous mutation load of HBsAg⁺ MCL patients was greater than that of HBsAg⁻ MCL patients. HDAC1, TRAF5, FGFR4, SMAD2, JAK3, SMC1A, ZAP70, BLM, CDK12, PLCG2, SMO, TP63, NF1, PTPR, EPHA2, RPTOR and FIP1L1 were significantly enriched in HBsAg⁺ MCL patients.

Few data are known regarding the use of interim positron emission tomography (iPET) after the first two cycles (iPET2) of chemotherapy in treatment-naïve classical Hodgkin lymphoma (cHL) in routine clinical practice, and about the real-life adoption of intensification strategies for iPET positive patients. We conducted a multicenter retrospective study on cHL to investigate the use of iPET in the real-life setting, its prognostic role and outcomes of patients early shifted to intensification. Six hundreds and forty-one patients were enrolled (62% had advanced stage). iPET2 was positive in 89 patients (14%) including 8.7% and 17% early and advanced stage patients, respectively (p = 0.003). Among iPET 2 positive cases treatment was immediately modified in 19 cases; in 14 cases treatment was modified after an additional positive iPET4. Overall 56 iPET2 positive patients never received intensified therapies. Most frequently used intensified therapy was autologous stem cell transplantation followed by BEACOPP. After a median follow-up of 72 months, the 5-year progression-free survival (PFS) was 82% with iPET2 positive patients showing a worse PFS compared with iPET2 negative cases: 31% versus 85%. Focusing on advanced stage patients with a positive iPET2, the 5-year PFS was 59% for patients shifted to intensified therapy at any time point versus 61% for patients who never received intensified therapy. Our study confirmed the higher curability of naïve cHL patients in a real-world setting, and the prognostic role of iPET2 in this setting. A poor adherence to response-adapted strategy which however did not translate into a difference in patient outcomes.

Cassin R, Rampi N, Fidanza C, et al. Reply to “successful early use of anti-SARS-CoV-2 monoclonal neutralizing antibodies in SARS-CoV-2 infected hematological patients—A Czech multicenter experience”: A case series of SARS-CoV-2 Omicron infection and aggressive lymphoma in the Sotrovimab era. Hematol Oncol. 2023; 41 (1):213-217. https://doi.org/10.1002/hon.3079.

In the article, the first name of the third author was abbreviated. The correct author name is Cecilia Anna Fidanza instead of Fidanza C.

We apologize for this error.

Classical Hodgkin lymphoma is a lymphoproliferative disease with a good prognosis mainly seen in young people. Nevertheless secondary malignancy, cardiac disease and infertility may affect the long survivors with significant impact on quality of life, morbidity and overall survival. In the last decades several treatment strategies were evaluated to reduce the toxicity of first line treatment such as avoiding radiotherapy or its reduction in terms of dosage and extension. Many trials including interim Positron Emission Tomography evaluation fail to compare efficacy between combined modality treatment versus chemotherapy alone in particular in early stage disease. In this review we analyze which subset of patients could take advantage from proton therapy in terms of toxicity and cost effectiveness.

Since its inception over 4 decades ago, the International Conference on Malignant Lymphoma (ICML) has steadily grown to become the leading international forum for lymphoma experts. Now with a biennial occurrence and more than 3000 participants, the ICML provides a unique opportunity for lymphoma clinicians, healthcare workers and scientists to come together and discuss novel data gleaned from discovery science, translational research, and clinical research efforts. Many pivotal findings in the lymphoma research community were first reported at ICML meetings and some of these have driven practice-changing approaches in lymphoma patient care.

As lymphoma scientists working at the Institute of Oncology Research in Bellinzona, Switzerland, we are proud to be involved in the ICML. In collaboration with Women in Lymphoma, we are excited to present to you our selected Discovery Science highlights from the 17th ICML meeting. Our aim is for these highlights to complement the clinical take-home messages presented at the 17-ICML highlights session and as such champion the importance of collaborative research which combines the expertise of clinical and non-clinical investigators, for the effective prevention, diagnosis and treatment of lymphomas.

Over the years, the work of a multitude of lymphoma researchers together with the emergence of new technologies, have resulted in a richer understanding of the molecular features that initiate and support lymphomagenesis. Laura Pasqualucci (New York, USA), is aptly recognised as having made seminal contributions to our understanding of lymphoma biology and as such, a number of her team's findings featured in her Meet the Professor session,¹ which focused on the role of the germinal center (GC) in the genesis of lymphomas. After giving an authoritative overview of the genetic, epigenetic and microenvironmental perturbations involved in the pathogenesis of lymphomas originating from the GC, she explained how she and Riccardo Dalla-Favera (New York, USA), embarked on opening the “big black box” that is the non-coding human genome to better study GC-derived lymphomas. Their investigations revealed that superenhancers (SE) were frequently hypermutated in diffuse large B cell lymphomas (DLBCLs). Over 90% of DLBCLs were found to harbor at least two mutations within SE regions thus indicating a selective pressure to acquire mutations in these regulatory domains. The activation induced cytidine deaminase, AID, was identified as a central player in the introduction of these mutations and the SE of key genes involved in lymphomagenesis were among those targeted. Working in the lab of Riccardo Dalla-Favera and Laura Pasqualucci, Elodie Bal, whose investigations uncovered this frequent targeting of SE in DLBCL, described her findings in more detail in two different sessions, Epigenetic mechanisms and targeted therapies in B- and T-cell lymphomas and Lymphoma