Acta Haematologica最新文献

Introduction: Leukemia is a group of diseases caused by malignant hematopoietic stem cell clones. Leukemia ranks 13th in incidence and 10th in mortality, according to the Global Cancer Statistics 2022.

Methods: We computed the prevalence, incidence, mortality, and disability-adjusted life years (DALYs) using the Global Burden of Disease (GBD) 2021 data. We rigorously tested the time trend from 1990 to 2021 using Joinpoint regression analysis. This method facilitates the calculation of annual percentage change (APC), average annual percentage change, and 95% confidence interval (CI).

Results: In 2021, the age-standardized prevalence rate (ASPR), age-standardized incidence rate (ASIR), age-standardized mortality rate (ASMR), and age-standardized DALYs rate of leukemia were 21.07 per 100,000 people (95% CI: 17.65-23.61), 5.63 per 100,000 people (95% CI: 4.83-6.17), 3.89 per 100,000 people (95% CI: 3.34-4.25), and 136.94 per 100,000 people (95% CI: 111.89-153.71), respectively. These figures have been obtained globally. Gender comparisons show that men have a higher burden of disease. The 90-94 age-group has the highest global prevalence of leukemia, with the prevalence increasing with age. Age-standardized DALYs rates, ASMR, and ASIR demonstrated a general declining trend from 1990 to 2021. The results of the Joinpoint analysis showed that between 2019 and 2021, there was a drop in the global ASPR (APC = -2.68%; 95% CI: -4.76% to -0.54%; p = 0.017), ASIR (APC = -2.46%; 95% CI: -3.33% to -1.59%; p < 0.001), and ASMR (APC = -1.87%; 95% CI: -2.75% to -0.99%; p < 0.001).

Conclusion: The results indicated an overall decrease in the burden of leukemia. These results provide important epidemiological data for the development of novel treatments.

Background: The advent of molecularly cloned hematopoietic growth factors (rhuG-CSF and rhuGM-CSF) enhanced the safety in treating acute myeloid leukemia (AML) by reducing the duration of neutropenia and thus decreasing the risks for infection. However, early in vitro studies demonstrated leukemia cell proliferation in response to these agents, raising reasonable concerns related to whether these factors can cause or contribute to relapse or progression of AML.

Summary: Clinical studies using recombinant myeloid hematopoietic growth factors have supported their safety, as there is little or no clear evidence for an association with an increased risk of relapse in AML in patients, regardless of the hematopoietic growth factor used, or whether the setting of AML is newly diagnosed or relapsed/refractory. One exception may be in the pediatric population, though this effect might reflect a different isoform of the G-CSF receptor expressed on the AML cells, as this truncated receptor is also seen in severe congenital neutropenia and aplastic anemia and underlies the increased myeloid malignancies that develop in these diseases after long-term exposure to growth factors.

Key messages: The current data support the use of rhuG-CSF and rhuGM-CSF in most patient populations with AML. Future studies should explore the factors influencing hematopoietic growth factor sensitivity in AML subpopulations to guide therapeutic decisions.

Introduction: Sarcopenia, defined by reduced muscle strength, mass, and performance, presents a significant challenge in cancer care due to its impact on treatment outcomes, quality of life, and survival. This study aimed to assess its prevalence in newly diagnosed lymphoma patients.

Methods: Adults planned for first-line anthracycline-based chemotherapy were enrolled and screened for sarcopenia before treatment. Sarcopenia was defined by the European guidelines (EWGSOP2) using low muscle strength (hand-grip), low muscle mass (DXA), and low physical performance (gait speed).

Results: Sixty-nine patients (mean age 57, 19 women) were included. Six patients (9%) had low hand-grip strength, 15 (22%) had low muscle mass, and 4 (6%) demonstrated low gait speed. Two patients met the criteria for sarcopenia, with one having severe sarcopenia.

Conclusion: Sarcopenia prevalence was 3%, but 22% had low muscle mass, suggesting muscle strength alone may not be an optimal screening tool for lymphoma patients.

Introduction: Acquired factor X (FX) deficiency is a rare coagulopathy. Occurrences in plasma cell dyscrasias (PCDs) independent of amyloid light-chain amyloidosis are exceedingly rare.

Case presentation: This case report presents a rare occurrence of acquired FX deficiency in a patient with multiple myeloma (MM) without concomitant amyloidosis. The patient, a 64-year-old male with prior diagnosis of smoldering MM, presented with abdominal pain and chronic bloody diarrheas and was diagnosed with absolute FX deficiency. Despite initial suspicion of amyloidosis, subsequent investigations ruled out its presence. Treatment with anti-myeloma therapy and supportive measures resulted in the normalization of coagulation parameters.

Conclusion: This case underscores the importance of considering acquired FX deficiency in PCD patients presenting with coagulopathy even in the absence of amyloidosis.

Introduction: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia that has become highly curable with advances in targeted therapy. However, central nervous system (CNS) involvement is exceedingly rare in APL and presents significant therapeutic challenges due to the limited penetrance of standard therapies across the blood-brain barrier (BBB). While APL is traditionally managed without chemotherapy, cases with CNS involvement require a multimodal approach for effective disease control.

Case presentation: We present a unique case of a 31-year-old male with de novo APL and CNS involvement at the presentation, including a clival mass. The patient was successfully treated with a combination of systemic chemotherapy, intrathecal chemotherapy, and craniospinal irradiation, leading to durable remission.

Conclusion: This case highlights the rarity of CNS involvement in APL and underscores the importance of a multidisciplinary approach in its management. Additionally, it emphasizes the need to address logistical barriers to treatment to achieve optimal patient outcomes.

Introduction: Mind-body interventions (MBIs) are therapeutic practices that target the interactions between cognitive, emotional, and physiological systems to influence health outcomes. Previously, we demonstrated that MBI prolonged lymphocyte doubling time and treatment-free survival (TFS) in treatment-naïve chronic lymphocytic leukemia (CLL) patients during the watch-and-wait phase. In this follow-up study, we investigated the long-term effects of MBI on TFS after the intervention ceased.

Methods: Sixty participants from the initial study (34 who received intervention vs. 26 controls) were followed for an additional period of 20 months. TFS was assessed from the end of the intervention to the initiation of CLL therapy or death, using Kaplan-Meier analysis and the log-rank test.

Results: By the end of the follow-up, 9 participants who previously received MBI and 6 controls initiated CLL treatment. No significant difference in TFS was found between the groups (log-rank test, p = 0.65).

Conclusion: While MBI provided a clear advantage as long as it continued, our follow-up analysis suggests this effect diminishes after the intervention ends. Continuous or repeated MBI may be necessary for sustained improvements in TFS.

Introduction: Immunotherapy in DLBCL (diffuse large B-cell lymphoma) disease has become an active research area with great value and potential. However, bibliometric research in this area is still sparse. Through bibliometric analysis, we aimed to visualize the research hot spots and trends of immunotherapy in DLBCL disease to help understand the future development of basic and clinical research.

Methods: The Web of Science Core Collection database was searched for articles and reviews related to the immunotherapy of DLBCL from 2004 to 2024. VOSviewers, CiteSpace, and the R package "bibliometrix" were used to conduct the bibliometric analysis.

Results: A total of 662 articles were included. The number of immunotherapy treatments in DLBCL increased year by year. The publications came from 55 countries, led by the USA and the People's Republic of China, and 1,349 institutions, with the leading research institutions being The University of Texas MD Anderson Cancer Center and Memorial Sloan Kettering Cancer Center. Leukemia & Lymphoma is the journal with the most research, and Blood is the journal with the most co-citations. We identified 4,833 authors, among which Young and Ken H. had the most significant articles, while Neelapu S.S. had the largest number of co-citations. After analysis, the most common keyword is CAR T (CAR T cells: chimeric antigen receptor T-cell immunotherapy, a current and developing area of research.

Conclusions: This is the first bibliometric study to comprehensively summarize research trends and advances in immunotherapy in DLBCL disease. This information will provide a reference for researchers and healthcare providers in immunotherapy research by clarifying recent research frontiers and hot spots such as CAR T cells, bispecific antibodies, and so on.

Introduction: High-dose therapy with melphalan followed by autologous stem cell transplant in the upfront setting (upfront ASCT) has significantly improved clinical outcomes of myeloma patients and become the standard of care for the past 30 years. However, with the advent of modern induction therapy, the role of upfront ASCT approach has been called into question. Several prospective studies have examined whether continuing with triplet therapy as consolidation with optional ASCT at relapse (triplet-alone) could result in comparable outcomes.

Methods: This was a systematic review and meta-analysis of randomized controlled trials comparing upfront ASCT versus triplet-alone approach among myeloma patients treated with triplet therapy, which included two novel agents and a corticosteroid, as induction. Cochrane Library, PubMed and conference proceedings were searched. Primary outcome was overall survival (OS). Secondary outcomes included progression-free survival (PFS), safety, and second primary malignancies (SPM). Subgroup analysis was conducted for high-risk cytogenetics.

Results: Our search yielded three trials, conducted between 2010-2018, including 1,737 patients. Two trials evaluated bortezomib plus lenalidomide (VRd) induction and the third study tested carfilzomib plus lenalidomide (KRd) induction. Maintenance was given in all trials to both arms. There was no difference in OS between the arms; the pooled OS in all patients and those with high-risk cytogenetics was hazard ratio (HR) 1.03 (95% CI, 0.85-1.26; I2 = 0%; 1,737 patients, 3 trials) and 0.85 (95% CI, 0.59-1.23; I2 = 0%; 222 patients, 2 trials), respectively. The pooled PFS for upfront ASCT versus triplet-alone was significantly improved in all the patients and in the high-risk cytogenetics subgroup, HR 0.67 (95% CI 0.59-0.76; I2 = 0%; 1,737 patients, 3 trials) and HR 0.59 (95% CI: 0.44-0.7; I2 = 0%; 306 patients, 3 trials), respectively. The risk of any grade 3-4 adverse events was higher in the upfront ASCT arm versus triplet-alone approach (relative risk = 1.17 [95% CI, 1.12-1.23; 1,737 patients]). The risk of secondary malignancies was reported in all three trials and was comparable between both arms. Two trials reported on secondary myeloid neoplasms, which were significantly higher among upfront ASCT arm versus triplet-alone approach, OR 9.7 (1.8-52.25, I2 = 0%, 1,422 patients).

Conclusion: Although upfront ASCT approach, in the era of triplet therapy, resulted in a significantly longer PFS among all patients, this did not translate into a survival benefit, regardless of cytogenetic risk. Upfront ASCT was associated with an increased rate of secondary myeloid neoplasms. In the current plethora of innovative therapies, the role of upfront ASCT is debatable.

Introduction: Acute myeloid leukemia (AML) represents a significant burden for patients and their families, and to the healthcare system. This study estimated the total cost of illness associated with newly diagnosed AML patients in Canada.

Methods: The economic burden of AML was estimated using an incidence-based model, analyzing different types of AML cases in Canada. Direct and indirect costs were calculated using scientific literature and Canadian clinical experts' inputs. Patients were categorized depending on their eligibility for intensive chemotherapy (fit and unfit patients) as well as according to age and cytogenetic markers.

Results: The total average cost of AML per patient is estimated to be CAD 178,073 with a cost of CAD 210,983 and CAD 145,163 for fit and unfit patients, respectively. The costs related to treatment represent half of the total average cost (52%), followed by hematopoietic stem cell transplant (23%), best supportive care (16%), productivity loss (6%), and wastage (4%).

Conclusion: For patients with AML, the costs associated with fit patients are higher than unfit patients. Hospitalization and best supportive care costs are key cost drivers for the total costs of fit and unfit patients, respectively. This study highlights that AML is associated with a significant economic burden in Canada.

Background: Lymphomas are a diverse group of disorders characterized by clonal proliferation of lymphocytes. While definitive diagnosis relies on histopathology, immunohistochemical, molecular and genomic analyses, imaging modalities including positron emission tomography/computed tomography (PET/CT), computed tomography (CT), and magnetic resonance imaging (MRI) are essential in diagnostic processes and management. Imaging aids in detecting suitable biopsy sites, assessing disease extent, evaluating treatment response, and detecting recurrence. However, accurate diagnosis and staging remain challenging due to tumor heterogeneity, inter-observer variability, and technical imaging issues.

Summary: Artificial intelligence (AI), particularly deep learning (DL) models, is transforming lymphoma imaging by enabling automated detection, segmentation, and classification across PET/CT, CT, and MRI modalities. Key applications include automated metabolic response assessment and total metabolic tumor volume quantification in PET/CT, lymph node segmentation and classification in CT, and improved detection of central nervous system involvement in MRI. Despite promising results, significant challenges limit widespread clinical adoption, including variability in imaging protocols affecting model generalizability, reliance on small retrospective datasets, lack of model interpretability, and difficulties integrating AI tools into existing clinical workflows.

Key messages: (1) DL applications can automate detection, segmentation, and classification in lymphoma imaging, improving diagnostic accuracy and reducing inter-observer variability across PET/CT, CT, and MRI modalities. (2) Challenges in DL adoption include validating model performance across diverse imaging protocols, addressing data biases, and ensuring generalizability to real-world clinical settings. (3) Integrating AI into clinical workflows requires careful validation to ensure safety, consistency, and alignment with existing diagnostic and treatment standards.