Acta Haematologica最新文献

{"title":"Concordance between Tympanic and Enteral Temperature for Early Fever Detection in Hematologic Intensive Care Unit Patients: A Prospective Study.","authors":"Florian Bouclet, Juliette Pénichoux, Sorina-Dana Mihailescu, Stéphanie Seris, Lucie Burel, Doriane Richard, Sabine Collard, Emmanuelle Jennet, Elisabeth Bertrand, Mustafa Alani, Sydney Dubois, Anne-Lise Ménard, Vincent Camus, Aspasia Stamatoullas, Pascal Lenain, Nathalie Contentin, Stéphane Leprêtre, Hervé Tilly, Emilie Lemasle, Hélène Lanic, Mikael Daouphars, Louis-Ferdinand Pépin, Sébastien Moussay, Fabrice Jardin","doi":"10.1159/000544693","DOIUrl":"10.1159/000544693","url":null,"abstract":"<p><strong>Introduction: </strong>Early detection of fever is crucial in neutropenic patients. Lack of precision in body temperature measurement can occur with peripheral site measurements. Furthermore, intermittent temperature monitoring may result in a delay in fever detection.</p><p><strong>Methods: </strong>We conducted a prospective study in hematologic intensive care unit (HICU) patients receiving autologous stem cell transplant or intensive chemotherapy in order to compare tympanic and enteral temperature measured by an ingested electronic pill (BodyCap®, France).</p><p><strong>Results: </strong>Twenty-six patients ingested at least one capsule with a total of 218 days of tympanic and enteral simultaneous measurement. In 12% of these measurements, we identified a difference over 0.5°C between the left ear versus right ear measurements. Enteral temperature was usually higher than the tympanic one (p < 0.0001) with 14% of the measurements that were discordant with higher enteral temperature and 1.9% that were discordant with higher tympanic temperature. Febrile episodes were detected with the ingestible pill on average almost 24 h earlier than with the tympanic measurement. Early capsule evacuation occurred in 20.4% of the cases, mainly because of diarrhea, a frequent adverse event in these patients. Patients were satisfied with capsule ingestion according to a questionnaire.</p><p><strong>Conclusion: </strong>In this prospective trial (TEMPET), we demonstrated that enteral temperature measured by an ingested pill is feasible in HICU and detects fever earlier than tympanic routine measurements. Digestive symptoms related to hematological disease and/or treatments are limiting factors for its generalized usage.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"113-124"},"PeriodicalIF":1.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143957940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute Promyelocytic Leukemia Presenting with Cranial Nerve Involvement and Clivus Mass.","authors":"Ananya Chandrika Nanduri, Farah Ashraf, Swe Swe Hlaing, Michael Brister, Tapan M Kadia, Naveen Pemmaraju, Tulin Budak-Alpdogan","doi":"10.1159/000545444","DOIUrl":"10.1159/000545444","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Case presentation: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"221-227"},"PeriodicalIF":1.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143750660","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emicizumab and Acquired Hemophilia A Secondary to Multiple Myeloma.","authors":"Florence Cuschera, Marie-Christiane Vekemans, Cedric Hermans","doi":"10.1159/000545442","DOIUrl":"10.1159/000545442","url":null,"abstract":"<p><strong>Introduction: </strong>Acquired hemophilia A (AHA) is a severe bleeding disorder, sometimes linked to plasma cell dyscrasias. In this context, emicizumab, a bispecific antibody, provides stable hemostasis by mimicking factor VIII (FVIII), offering convenience and flexibility compared to bypassing agents. Moreover, targeted anti-myeloma therapy directly addresses the underlying plasma cell disorder, potentially achieving better and more durable control of AHA than conventional immunosuppressive therapy, while reducing its associated adverse effects.</p><p><strong>Case presentation: </strong>We describe the successful management of AHA secondary to multiple myeloma (MM) using emicizumab and targeted anti-myeloma therapy. The patient initially responded to bortezomib-dexamethasone but required teclistamab due to disease progression. Emicizumab maintained hemostatic stability, allowing time for effective MM management.</p><p><strong>Conclusion: </strong>Emicizumab, in conjunction with targeted myeloma treatment, represents a promising strategy to improve outcomes for AHA patients with MM. This approach is particularly advantageous for older patients with multiple comorbidities, who face elevated risks of thrombotic, bleeding, and infectious complications.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"233-240"},"PeriodicalIF":1.1,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143959199","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Identification of Needs of Patients with Multiple Myeloma: A Questionnaire-Based Study in Israeli Patients.","authors":"Ilana Levy Yurkovski, Shira Ben-Dov, Varda Shoam, Micha Yuz, Noa Lavi","doi":"10.1159/000547025","DOIUrl":"10.1159/000547025","url":null,"abstract":"<p><p><p>Introduction: Multiple myeloma is an incurable chronic malignant disease. The disease itself and its treatment impair quality-of-life (QoL), yet there is no data regarding the biopsychosocial needs of patients in the era of new treatments. In the current study, we aimed to identify the biopsychosocial needs of patients with multiple myeloma.</p><p><strong>Methods: </strong>This is a descriptive study on patients with multiple myeloma in Israel in 2024. The information was based on a questionnaire examining physical, psychological, and social needs filled out by myeloma patients. We analyzed the main impairments of QoL and what affected them, the main supporter in dealing with the disease, psychosocial needs reported by the patients and the difficulties in dealing with such difficulties.</p><p><strong>Results: </strong>The main symptom reported by multiple myeloma patients was fatigue. The number of treatment lines worsened QoL (unstandardized coefficient: 0.987, 95% CI: 0.284; 1.691, p = 0.006). The patient's partner mostly helped in dealing with the disease (72.7%). The most desired type of support was assistance in accessing rights (median 5, interquartile range 3-5); however, one-third did not use the support services offered to them. A total of 48% the patients talked to their doctor about the struggle and the accompanying difficulties.</p><p><strong>Conclusion: </strong>Myeloma patients report various impairments in the biopsychosocial components of QoL. Although supportive services are offered, adjustments must be made to optimally meet patients' needs. Further studies should test the effectiveness of different interventions on the biopsychosocial components of the QoL of these patients in the era of new drugs. </p>.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"338-344"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12258863/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144482830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chronic Graft-versus-Host Disease: A Review of Current Treatments beyond Second-Line and Emerging Therapies.","authors":"Aseel Saadeh, Dayana Jibrin, Michael Haddadin","doi":"10.1159/000550265","DOIUrl":"10.1159/000550265","url":null,"abstract":"<p><strong>Background: </strong>Chronic graft-versus-host disease (cGVHD) remains a major cause of late morbidity and non-relapse mortality following allogeneic hematopoietic cell transplantation. Early identification and intervention are critical to improving outcomes. Corticosteroids continue to serve as the first-line therapy; however, treatment-refractory cases are common and associated with poor outcomes. Ruxolitinib has become the standard second-line agent, yet beyond ruxolitinib, treatment selection varies widely due to a lack of comparative data and standardized sequencing strategies. A comprehensive review of key therapeutic trials in chronic GVHD was conducted, with a focus on second-line treatments and beyond. Emerging and investigational therapies were also included through analysis of recent literature and ongoing studies.</p><p><strong>Summary: </strong>There is currently no established sequencing of agents beyond second-line therapies. The approval of belumosudil, axatilimab, and ibrutinib has expanded therapeutic options, with each agent offering a unique mechanism of action and demonstrating promising organ-specific response rates.</p><p><strong>Key messages: </strong>The therapeutic landscape for chronic GVHD is evolving, with several newer agents beginning to demonstrate utility in third-line settings. However, the selection of third-line agents remains largely dependent on clinical judgment, prior treatment history, and the specific organs involved. There is a continuing and critical need for well-designed, comparative trials to establish optimal treatment strategies.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"594-603"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145958551","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patients with Prior Cerebral Venous Sinus Thrombosis Presenting to the Emergency Department with Neurological Symptoms: The Yield of Neuroimaging.","authors":"Evgeny Grishin, Ivan Budnik, Orly Efros, Omri Cohen, Gili Kenet, Sarina Levy-Mendelovich, Assaf Arie Barg","doi":"10.1159/000549917","DOIUrl":"10.1159/000549917","url":null,"abstract":"<p><p><p>Introduction: Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening condition. Despite advances in diagnostic imaging and therapeutic strategies, data regarding risk factors for CVST progression among patients remain limited.</p><p><strong>Methods: </strong>The retrospective cohort study aimed to evaluate the yield of neuroimaging and to assess risk factors for CVST progression among patients presenting to the emergency department (ED) after a prior CVST diagnosis. We collected data from the hospital's electronic medical records on patients diagnosed with CVST at our tertiary care center between January 2002 and April 2023. For patients who had subsequent ED visits related to their initial CVST diagnosis, data regarding demographics, clinical presentation, imaging outcomes, and alterations in therapeutic management were retrieved.</p><p><strong>Results: </strong>Our initial cohort included 251 patients diagnosed with CVST. Of these, 107 (43%) patients returned to the ED with symptoms potentially related to CVST. Headache was the most common presenting symptom (59%), and imaging was performed in 71% of relevant ED visits. Thrombus progression was observed in only 6% of cases. No significant associations were found between demographic factors, clinical presentation, anticoagulation status, and neuroimaging findings. Among patients whose initial CVST diagnosis occurred more than 2 years prior to ED evaluation, only those with thrombophilia experienced thrombus progression.</p><p><strong>Conclusions: </strong>Thrombus progression is a rare finding among patients with a history of CVST presenting to the ED with neurological complaints. Pediatric patients showed low rates of thrombotic worsening, suggesting a more judicious use of neuroimaging in this population. No significant risk factor was found to predict the risk of CVST progression. </p>.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"564-574"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12826760/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145740542","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paroxysmal Nocturnal Hemoglobinuria with Large Clones in Non-Hypoplastic Myelodysplastic Syndrome: Report of Two Cases.","authors":"Janine Briggeler-Mani, Emmanuel Häfliger, Annatina Sarah Schnegg-Kaufmann, Katarzyna Aleksandra Jalowiec, Nicola Andina, Nada Agbariah, Adrian Dante De Angelis, Bastien Grandjean, Linet Njue, Allam Ramanjaneyulu, Ulrike Bacher, Yara Banz, Naomi Azur Porret, Alicia Rovó","doi":"10.1159/000548287","DOIUrl":"10.1159/000548287","url":null,"abstract":"<p><strong>Introduction: </strong>Paroxysmal nocturnal hemoglobinuria (PNH) clones are frequently found in hypoplastic myelodysplastic syndromes (hMDS), though less commonly than in aplastic anemia. In contrast, the coexistence of hemolytic PNH with large clones and classical, hypercellular MDS (non-hMDS) is rare and likely underrecognized in clinical practice. Since 2014, 229 MDS patients have been seen at our department. Here, we report two cases with this association and discuss their particular diagnostic and treatment challenges.</p><p><strong>Case presentations: </strong>The first case is a 68-year-old woman with a hemolytic PNH of 59 years duration. We first saw her in June 2021; she had pancytopenia, with values stable over the past 25 years. After a complete work-up, MDS with low blasts and SF3B1 mutation was diagnosed. She was subsequently diagnosed with symptomatic pulmonary hypertension, and in 2023, she started therapy with ravulizumab, achieving good disease control. The second case concerns a 76-year-old man diagnosed with MDS at age 74. One year later, his anemia worsened, and hemolytic PNH with large clones was diagnosed. The patient showed initial benefit from ravulizumab, and he was later switched to pegcetacoplan, which led to effective disease control.</p><p><strong>Conclusion: </strong>We want to emphasize the importance of assessing PNH clones in the diagnosis of non-hMDS, especially in cases with significant anemia. Regarding PNH treatment in such patients, we found that they are underrepresented in studies investigating complement inhibitor. However, standard doses recommended for PNH appear effective and safe regardless of the underlying disease.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"375-382"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145022547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Approach to Hairy-Cell Leukemia in the New Therapeutic Era with Special Emphasis on Age and Comorbidities.","authors":"Ilana Levy Yurkovski, Tamar Tadmor","doi":"10.1159/000547722","DOIUrl":"10.1159/000547722","url":null,"abstract":"<p><p><p>Background: Hairy-cell leukemia (HCL) is a rare chronic hematologic malignancy, generally presenting with pancytopenia, relative lymphocytosis, monocytopenia, and splenomegaly. Diagnosis is based on typical bone marrow evaluation with the BRAF-V600E mutation being present in almost 100% of cases of classical HCL. Summary: Treatment usually involves the use of purine analogs (PAs) as first-line therapy. Novel targeting therapies have recently been included in the treatment of therapy-naive HCL such as PA combination with anti-CD20, BRAF inhibitors alone, or combined with anti-CD20. In relapse/refractory disease, other novel agents were studied as BRAF and MEK inhibitors, Bruton tyrosine kinase, or BCL2 inhibitors: all showed encouraging results. Most clinical trials and guidelines do not specify what the optimal approach is for patients with HCL in special situations as elderly population above 80 years old, very young patients below 40 years old, pregnant women, and when leukemia is presented with other comorbidities as active infection or vasculitis. Key Messages: In this current manuscript, we summarized our approach to HCL in the era of novel agents with special emphasis on age and comorbidities. </p>.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"477-486"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12503663/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144783198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Venetoclax-Based Regimen in Refractory or Relapsed Pediatric Acute Lymphoblastic Leukemia.","authors":"Lin Zhang, Zhixiao Zhang, Aidong Lu, Yueping Jia, Lin Huang, Leping Zhang, Huimin Zeng","doi":"10.1159/000547080","DOIUrl":"10.1159/000547080","url":null,"abstract":"<p><strong>Introduction: </strong>This study investigated the efficacy and survival of pediatric refractory or relapsed (R/R) acute lymphoblastic leukemia (ALL) treated with a venetoclax (VEN)-based regimen.</p><p><strong>Methods: </strong>Children with R/R ALL treated with a VEN-based regimen at Peking University People's Hospital from December 1, 2018, to January 15, 2024, were included in this study. Complete remission (CR) or complete remission with incomplete recovery of blood count (CRi) rates and objective response rates (ORRs) were analyzed.</p><p><strong>Results: </strong>Twenty-two children with R/R ALL were included in this study. The median duration of VEN treatment per cycle was 21 (7-28) days, and the median VEN dose was 100 (50-300) mg/day. Following a cycle of VEN-based therapy, 17 children (77.3%) achieved CR/CRi/morphological leukemia-free state (MLFS), including 8 cases (8/17) with negative MRD. The ORR in the children with B-cell acute lymphoblastic leukemia (B-ALL) (n = 9) and T-cell acute lymphoblastic leukemia (T-ALL) (n = 8) was 75% and 80%, respectively. Patients with early T-cell precursor (ETP) ALL (n = 6) achieved MRD-negative remission, and one KMT2A::USP2-positive child achieved MLFS after receiving a VEN-based regimen. For the relapsed patients, the median overall survival (OS) was 1,371 days. For the refractory patients, the median OS was unreached. For T-ALL patients, the median OS was 1,371 days. For the patients with B-ALL, the median OS was 543 days. All patients had hematologic adverse reactions within an acceptable range.</p><p><strong>Conclusion: </strong>Children with R/R ALL who received the VEN-based regimen achieved a high remission rate with an acceptable safety profile. Significantly, the VEN-based regimen was effective in patients with R/R ETP with MRD-negative results while also proving beneficial for KMT2A-rearranged, highlighting VEN-chemotherapy as a treatment option for remission.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"280-286"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144590221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advances in Acute Lymphoblastic Leukemia Review Series.","authors":"Jayastu Senapati, Nicholas J Short","doi":"10.1159/000547659","DOIUrl":"10.1159/000547659","url":null,"abstract":"","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"5-7"},"PeriodicalIF":2.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12818103/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144740816","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}