Acta Haematologica最新文献

{"title":"Disseminated Mucormycosis and T-Cell-Depleted Allogeneic Stem Cell Transplantation: An Unusual Case Study.","authors":"Oana Diana Dragoi, Mili Shah, Victoria Potter, Daniele Avenoso, Pramila Krishnamurthy, Alireza Abdolrasouli, Silke Schelenz, Julie Chandra, Varun Mehra","doi":"10.1159/000540640","DOIUrl":"10.1159/000540640","url":null,"abstract":"<p><strong>Introduction: </strong>Invasive fungal infections are a primary cause of morbidity and mortality in patients with haematological malignancies.</p><p><strong>Case presentation: </strong>We describe an unusual clinical and radiological presentation of invasive mucormycosis (IM) in a 69-year-old patient with relapsed acute myeloid leukaemia. The patient was diagnosed with disseminated IM with involvement of the central nervous system in an atypical location, lung, spleen, muscle, bone, and heart, after having completed induction and bridging chemotherapy to allogeneic haematopoietic stem cell transplant (HSCT). Her clinical presentation was atypical with mild neurological symptoms slowly progressing over 2 months and without appropriate signs of systemic inflammation. Mucorales was eventually confirmed from bronchoalveolar lavage and subdural collection.</p><p><strong>Conclusion: </strong>This report highlights the difficult challenges of managing disseminated IM in an immunocompromised patient, where close multidisciplinary specialist care enabled successful treatment, followed by T-cell-depleted allogeneic HSCT for a high-risk haematological malignancy.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"362-368"},"PeriodicalIF":1.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142016008","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":"Belantamab Mafodotin in Relapsed/Refractory AL Amyloidosis: Real-World Multi-Center Experience and Review of the Literature.","authors":"Eyal Lebel, Vladimir Vainstein, Paolo Milani, Giovanni Palladini, Tamir Shragai, Noa Lavi, Hila Magen, Miri Assayag, Irit Avivi, Moshe E Gatt","doi":"10.1159/000541594","DOIUrl":"10.1159/000541594","url":null,"abstract":"<p><strong>Introduction: </strong>Treatment for relapsed/refractory AL amyloidosis (AL) is an unmet need. The safety and efficacy of belantamab mafodotin (BLM) in multiple myeloma are known, whereas in AL data are limited.</p><p><strong>Methods: </strong>We report a multi-center cohort of AL patients receiving BLM, and review all previous data on BLM therapy in AL.</p><p><strong>Results: </strong>Twelve patients with a median of 3 (range 2-9) prior lines of therapy were included. The overall hematological response rate (ORR) was 75% (9/12), including 5 complete responses. Six of the 10 evaluable patients had organ responses. The median event-free survivals/overall survivals were 22.3 and 28.8 months, respectively. Grade 3 toxicities were mostly infections and keratopathy, occurring in 7/12 (58%). Hematological toxicities were rare. No grade 4/5 toxicities occurred. The review of the previous series reveals BLM provides an ORR of 60-83% with similar rates of corneal toxicity.</p><p><strong>Conclusion: </strong>BLM, being an off-the-shelf therapy, with acceptable toxicity even in frail patients, may be a valuable option in AL, with a high ORR, and a signal for durable responses and high-quality organ responses.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"419-426"},"PeriodicalIF":1.1,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142363859","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":"Haploidentical Allogeneic Hematopoietic Cell Transplantation following Two Courses of Venetoclax and Azacytidine Therapy in Patients over 55 Years Old with Acute Myelogenous Leukemia: Comment.","authors":"Hinpetch Daungsupawong, Viroj Wiwanitkit","doi":"10.1159/000542843","DOIUrl":"10.1159/000542843","url":null,"abstract":"","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-2"},"PeriodicalIF":1.7,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142765464","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":"Intake of Proton Pump Inhibitors Is Associated with a Shorter Time to First Treatment in Early-Stage Chronic Lymphocytic Leukemia.","authors":"Tamar Tadmor, Guy Melamed, Hilel Alapi, Sivan Gazit, Tal Patalon, Lior Rokach","doi":"10.1159/000541453","DOIUrl":"10.1159/000541453","url":null,"abstract":"<p><strong>Introduction: </strong>Proton pump inhibitors (PPIs) are one of the most widely used drugs worldwide [Gut Liver. 2017;11(1):27-37]. The use of PPI has become a common practice and is overprescribed for all patients with cancer including patients with hematological malignancies. In the current study, we aimed to explore retrospectively the effect of PPI, on time to first treatment (TTFT) in a large cohort of patients with chronic lymphocytic leukemia (CLL) who were under watch-and-wait approach.</p><p><strong>Methods: </strong>The cohort is based on anonymized data obtained from electronic medical records of Maccabi Healthcare Services (MHS) members, who is the second-largest healthcare organization in Israel, with 2.5 million insured patients, and received a diagnosis of CLL during this period.</p><p><strong>Results: </strong>Our cohort included 3,474 patients with CLL who are treatment-naïve, and the median follow-up was 1,745 days (602-3,700). A total of 1,061 patients (30.5%) received a PPI agent, for a minimum of 3 months during the watch-and-wait period. The intake of PPI was found to be associated with a shorter TTFT: among PPI users, the 10-year treatment-free ratio is 79.2%, while among non-PPI users it is 90.6%.</p><p><strong>Conclusion: </strong>Routine use of PPI in CLL patients may negatively impact their clinical course. Biology of this primary observation requires further investigation.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-7"},"PeriodicalIF":1.7,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142339058","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":"BOSUTINIB TREATMENT OF CHRONIC MYELOID LEUKEMIA IN LOMBARDY.","authors":"Alessandra Iurlo,Cristina Bucelli,Tamara Intermesoli,Chiara Elena,Mariella D'Adda,Elena Agostani,Cristina Fiamenghi,Margherita Maffioli,Nicola Orofino,Francesca Lunghi,Angelo Gardellini,Maria Cristina Carraro,Alessandro Inzoli,Federica Gigli,Roberto Palazzolo,Vanda Bertolli,Daniele Cattaneo,Ester Maria Pungolino,Carlo Gambacorti-Passerini","doi":"10.1159/000540572","DOIUrl":"https://doi.org/10.1159/000540572","url":null,"abstract":"Introduction Up to 30% of CML patients will require a therapeutic change during follow-up, due to intolerance and/or resistance to first-line TKI approach. In this context, bosutinib (BOS) has not only demonstrated its effica-cy, but also presents a favorable safety profile, without comorbid conditions representing an absolute contraindication to its use. Methods To gain further into BOS treatment in real-life, we conducted a retrospective analysis on the outcome of CML patients receiving BOS in 18 hematological centers, all belonging to the \"REL\" (Lombard Hematology Network). Results Of 546 regularly followed CML cases, a total of 132 patients were reported as being treated with BOS, most frequently (62.9%) in second line. Interestingly, most patients (63.6%) switched to BOS due to intol-erance to the previous TKI, while resistance to the last treatment was reported in the remaining 36.4% of patients. Despite a permanent discontinuation rate of 18.9%, over 80% of patients achieved at least an MMR and seven cases were able to attempt treatment-free remission. Conclusion Although in this survey BOS represented the preferred option especially in patients intolerant rather than resistant to previous TKIs, we confirmed that BOS represents a safe and effective therapeutic option be-yond first line in the real-life setting.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"16 1","pages":"1-13"},"PeriodicalIF":2.4,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142250221","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":"Outcomes of Fedratinib in routine Treatment of ruxolitinib-resistant or refractory patients with Primary and post-polycythemia vera or essential thrombocythemia Myelofibrosis: A nationalwide retrospective study.","authors":"Adrian Duek,Alexandra Tzinman,Kira Maziuk,Assaf Levy,Martin Ellis,Galia Stemer,Adi Shacham Abulafia,Amos Cohen,Noa Lavi,Aaron Ronson,Andrey Braester,Shirley Shapira,Jonathan Canaani,Yulia Volchek,Ronit Leiba,Merab Leiba","doi":"10.1159/000540906","DOIUrl":"https://doi.org/10.1159/000540906","url":null,"abstract":"INTRODUCTIONIn recent years, fedratinib, a selective JAK2 inhibitor, has emerged as a potential therapeutic option for patients who have failed or are intolerant to ruxolitinib. Despite the promising results observed in clinical studies, real-world evidence from the United States and Europe suggests that the efficacy of fedratinib may be less conclusive. We report the characteristics, treatment patterns, and clinical outcomes of patients with myelofibrosis (MF) treated with fedratinib following ruxolitinib failure in Israel's clinical practice.METHODSThis retrospective patient chart review included adults with a physician-reported diagnosis of MF, who initiated fedratinib after discontinuing ruxolitinib. Descriptive analyses characterized patient characteristics, clinical outcomes, and treatment patterns from MF diagnosis through ruxolitinib and fedratinib treatment.RESULTSWe extracted data for 16 eligible patients. Approximately 62.5 % of the patients were female, and the median age was 77 (range, 63-85) years. The median duration of ruxolitinib therapy was 17 months (range 3-84 ) months. Before the initiation of fedratinib, the median spleen size by palpation was 15.5cm below the costal margin (range 4-22cm). After three months the median spleen size was 13cm below the costal margin (range 2-21 cm). Only two patients showed minimal improvement after six months, while three patients progressed, and two patients showed no change in the spleen size. The spleen response did not improve after 12 months of treatment. At this point, the median spleen size was 19 cm below the costal margin (range 2-30 cm). Regarding the MF-related symptoms, 43.75% (n =7) of patients reported some improvement, 37.5% (n =6) showed no changes, whereas 18.75% (n =3) of the population complained of worsening. Gastrointestinal toxicity was the most frequent adverse effect of the drug, while 31% of patients died.CONCLUSIONOur observations showed that in MF patients who have failed to ruxolitinib, the therapeutic value from fedratinib may be modest especially when exposure time to ruxolitinib was more than 12 months. We may hypothesize that earlier switching from ruxolitinib to fedratinib may yield a better result.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"15 1","pages":"1-11"},"PeriodicalIF":2.4,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142250121","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":"Beneficial effect of integrated nutritional interventions in patients with hematological diseases undergoing hematopoietic stem cell transplant.","authors":"Mimi Geng,Zihui Sun","doi":"10.1159/000541154","DOIUrl":"https://doi.org/10.1159/000541154","url":null,"abstract":"INTRODUCTIONThe nutritional status of patients undergoing hematopoietic stem cell transplantation (HSCT) is critically important. This study was aimed to assess the impact of comprehensive nutritional interventions on the well-being of individuals with hematological diseases who underwent HSCT.METHODSA total of 175 patients with hematological diseases who underwent HSCT were included, with 94 in the control group and 81 in the research group. Patients in the control group received standard nursing care, while those in the research group underwent integrated nutritional interventions. Nutritional status was evaluated using the mini nutritional assessment (MNA) and subjective global assessment (SGA), along with body measurements and serum levels of albumin, prealbumin, and hemoglobin.RESULTSThere were no significant differences in the proportion of malnourished patients evaluated by MNA or SGA between the control and research groups at admission. However, at discharge and 3 months post-discharge, fewer patients in the research group were malnourished compared to the control group, as assessed by both MNA and SGA. At admission, there were no significant differences in albumin, prealbumin, hemoglobin levels, weight, calf circumference (CC), triceps skinfold thickness (TSF), or subscapular skinfold thickness (SSF) between groups. However, at discharge and 3 months post-discharge, the levels of these indicators significantly decreased compared to those upon admission. Levels of albumin, prealbumin, and hemoglobin, as well as weight, CC, TSF, and SSF, were significantly higher in the research group than in the control group at both discharge and 3 months post-discharge.CONCLUSIONHSCT led to a decline in nutritional status among patients with hematological diseases, but integrated nutritional interventions effectively improved their nutritional status.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"39 1","pages":"1-14"},"PeriodicalIF":2.4,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142250122","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":"Pathophysiology of Acute Myeloid Leukemia.","authors":"Franziska Wachter, Yana Pikman","doi":"10.1159/000536152","DOIUrl":"10.1159/000536152","url":null,"abstract":"<p><strong>Background: </strong>Acute myeloid leukemia (AML) is a biologically heterogenous disease arising in clonally proliferating hematopoietic stem cells. Sequential acquisition of mutations leads to expanded proliferation of clonal myeloid progenitors and failure of differentiation, leading to fulminant AML.</p><p><strong>Summary: </strong>Here, we review the pathophysiology of AML with a focus on factors predisposing to AML development, including prior chemo- and radiation therapy, environmental factors, and germline predisposition.</p><p><strong>Key message: </strong>Increasing genomic characterization of AML and insight into mechanisms of its development will be critical to improvement in AML prognostication and therapy.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"229-246"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139477819","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":"Bortezomib and Vorinostat Therapy as Maintenance Therapy Post-Autologous Transplant for Non-Hodgkin's Lymphoma Using R-BEAM or BEAM Transplant Conditioning Regimen.","authors":"Leona A Holmberg, David G Maloney, Laura Connelly-Smith","doi":"10.1159/000533944","DOIUrl":"10.1159/000533944","url":null,"abstract":"<p><strong>Introduction: </strong>The success of autologous stem cell transplantation (ASCT) for treating non-Hodgkin's lymphoma (NHL) is limited by its high relapse rates. To reduce the risk of relapse, additional maintenance therapy can be added post-transplant. In a non-transplant setting at the time of initiation of this study, both bortezomib and vorinostat had been studied alone or in combination for some NHL histology and showed some clinical activity. At our center, this combination therapy post-transplant for multiple myeloma showed acceptable toxicity. Therefore, it seemed reasonable to study this combination therapy post-ASCT for NHL.</p><p><strong>Methods: </strong>NHL patients underwent conditioning for ASCT with rituximab, carmustine, etoposide, cytarabine, melphalan/carmustine, etoposide, cytarabine, melphalan. After recovery from the acute transplant-related toxicity, combination therapy with IV bortezomib and oral vorinostat (BV) was started and was given for a total of 12 (28-day) cycles.</p><p><strong>Results: </strong>Nineteen patients received BV post-ASCT. The most common toxicities were hematologic, gastrointestinal, metabolic, fatigue, and peripheral neuropathy. With a median follow-up of 10.3 years, 11 patients (58%) are alive without disease progression and 12 patients (63%) are alive.</p><p><strong>Conclusions: </strong>BV can be given post-ASCT for NHL and produces excellent disease-free and overall survival rates.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"300-309"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10590639","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":"Consensus Statements Highlight the Need of Harmonizing Chronic Lymphocytic Leukemia Management Worldwide.","authors":"Stefano Molica, Marco Rossi, David Allsup","doi":"10.1159/000533349","DOIUrl":"10.1159/000533349","url":null,"abstract":"<p><p>In addition to the European Society for Medical Oncology (ESMO) and National Comprehensive Cancer Network (NCCN) guidelines that are reference standards for the treatment of chronic lymphocytic leukemia (CLL) in Europe and the USA, several consensus statements, formulated by independent, multidisciplinary panels of specialists, have been developed to provide region-specific guidance for the management of CLL.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"257-259"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9944849","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}