Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-11-16DOI: 10.1159/000535275
Roberta Loveikyte, Yascha van den Berg, Andrea Elisabeth van der Meulen-de Jong, Lodewijk Thomas Vlasveld
{"title":"The Role of Hepcidin and an Oral Iron Absorption Test in Identifying the Root Cause of Iron-Restricted Anemia (Enter-Iron).","authors":"Roberta Loveikyte, Yascha van den Berg, Andrea Elisabeth van der Meulen-de Jong, Lodewijk Thomas Vlasveld","doi":"10.1159/000535275","DOIUrl":"10.1159/000535275","url":null,"abstract":"<p><strong>Introduction: </strong>Traditional iron parameters often fail to distinguish the cause of iron-restricted anemia in patients without an obvious underlying cause. We evaluated whether an oral iron absorption test (OIAT) and hepcidin measurement could be useful diagnostic tests in these patients.</p><p><strong>Methods: </strong>We retrospectively analyzed data extracted from medical records of all patients who underwent an OIAT and hepcidin measurement, noting subsequent clinical diagnosis. Δ Iron >15 µmol/L during the OIAT and a hepcidin level below the median (or suppressed ≤0.5 n<sc>m</sc>) were considered appropriate.</p><p><strong>Results: </strong>Thirty-nine adult patients were included in the study. Sixteen patients with adequate OIAT had suppressed hepcidin levels indicative of classical iron-deficiency anemia (IDA); 59% of patients had abnormal OIAT. In this group, most patients with low hepcidin levels had anemia associated with abnormalities in the gastrointestinal tract, whereas 83.3% patients with high hepcidin levels had iron-refractory iron-deficiency anemia (IRIDA), confirmed by genetic testing. Finally, transferrin/log ferritin ratio accurately identified patients with suppressed hepcidin: AUC 0.98 [95% CI: 0.95-1.02], p < 0.001.</p><p><strong>Conclusion: </strong>OIAT differentiates between classical IDA and other types of anemia caused by abnormalities in iron absorption or systemic iron availability. Additionally, elevated hepcidin in patients with oral iron malabsorption could indicate IRIDA.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"402-412"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11296559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136395726","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-11-14DOI: 10.1159/000534453
Fausto A Rios-Olais, Fernando Gil-Lopez, Analy Mora-Cañas, Roberta Demichelis-Gómez
{"title":"Tumor Lysis Syndrome Is Associated with Worse Outcomes in Adult Patients with Acute Lymphoblastic Leukemia.","authors":"Fausto A Rios-Olais, Fernando Gil-Lopez, Analy Mora-Cañas, Roberta Demichelis-Gómez","doi":"10.1159/000534453","DOIUrl":"10.1159/000534453","url":null,"abstract":"<p><strong>Introduction: </strong>Tumor lysis syndrome (TLS) occurs frequently during induction therapy for acute lymphoblastic leukemia (ALL). Patients are categorized into intermediate- or high-risk based on the lactate dehydrogenase (LDH) value and white blood cell (WBC) count, according to an expert panel, although no effort has been made to analyze TLS in ALL and its potential consequences.</p><p><strong>Methods: </strong>We retrospectively analyzed TLS, variables associated with its occurrence, and its impact on overall survival (OS) and mortality during induction in a cohort of ALL patients in their first induction regimen.</p><p><strong>Results: </strong>A total of 138 patients were included, 52.9% were male and the median age at diagnosis was 34 years. Most of them were treated with hyper-CVAD (39.1%) or a modified CALGB 10403 regimen (37.7%). TLS was identified in 42 patients (30.4%), and half of them fulfilled criteria for clinical TLS (C-TLS). Median OS was the lowest in C-TLS patients. An LDH 3 times greater than its upper laboratory normal (ULN) value and a WBC count equal to or greater than 50×109/L were associated with TLS development, and being male, hyperuricemia and an LDH 3 times greater than its ULN value were associated with C-TLS development. C-TLS and acute kidney injury were associated with excess mortality during induction.</p><p><strong>Conclusion: </strong>TLS was identified in almost one-third of ALL patients during induction therapy. Different thresholds for LDH value and WBC count as well as other variables could identify patients at risk of developing this complication, which is associated with shorter OS. C-TLS confers a higher risk for mortality during induction.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"391-401"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"107589942","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-09-13DOI: 10.1159/000533232
Tamar Tadmor, Jan Burger
{"title":"Chronic Lymphocytic Leukemia: Novel Perspectives - How to Teach an Old Dog New Tricks.","authors":"Tamar Tadmor, Jan Burger","doi":"10.1159/000533232","DOIUrl":"10.1159/000533232","url":null,"abstract":"","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"5-7"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10228899","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-10-13DOI: 10.1159/000533776
Anke Rihsling, Helena Simeunovic, Sergio Sanchez, Christine Henny, Sofia Lejon Crottet, Behrouz Mansouri Teleghani, Michael Daskalakis, Martin Müller, Luigi Raio, Alicia Rovó
{"title":"\"Don't Add Fuel to the Fire\"- Hyperhemolysis Syndrome in a Pregnant Woman with Compound Sickle Cell Disease/β0-Thalassemia: Case Report and Review of the Literature.","authors":"Anke Rihsling, Helena Simeunovic, Sergio Sanchez, Christine Henny, Sofia Lejon Crottet, Behrouz Mansouri Teleghani, Michael Daskalakis, Martin Müller, Luigi Raio, Alicia Rovó","doi":"10.1159/000533776","DOIUrl":"10.1159/000533776","url":null,"abstract":"<p><p>Hyperhemolysis syndrome (HHS) is a rare and severe posttransfusion complication characterized by the destruction of both recipient and donor red blood cells. The underlying mechanism of HHS is not fully understood and proper management can be difficult. Furthermore, there are few reports regarding HHS in pregnancy. We report on the development and management of HHS in a pregnant woman with known compound sickle cell disease (SCD)/β0-thalassemia and alloimmunization after transfusion of packed red blood cells (PRBC). We aim to raise awareness on this diagnostically challenging and life-threatening type of hemolysis with this report, and to stress the need to consider the diagnosis of HHS in SCD patients with progressive anemia despite PRBC administration.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"378-387"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41231534","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-09-28DOI: 10.1159/000533665
Hamed Shourabizadeh, Dionne M Aleman, Louis-Martin Rousseau, Arjun D Law, Auro Viswabandya, Fotios V Michelis
{"title":"Machine Learning for the Prediction of Survival Post-Allogeneic Hematopoietic Cell Transplantation: A Single-Center Experience.","authors":"Hamed Shourabizadeh, Dionne M Aleman, Louis-Martin Rousseau, Arjun D Law, Auro Viswabandya, Fotios V Michelis","doi":"10.1159/000533665","DOIUrl":"10.1159/000533665","url":null,"abstract":"<p><strong>Introduction: </strong>Prediction of outcomes following allogeneic hematopoietic cell transplantation (HCT) remains a major challenge. Machine learning (ML) is a computational procedure that may facilitate the generation of HCT prediction models. We sought to investigate the prognostic potential of multiple ML algorithms when applied to a large single-center allogeneic HCT database.</p><p><strong>Methods: </strong>Our registry included 2,697 patients that underwent allogeneic HCT from January 1976 to December 2017. 45 pretransplant baseline variables were included in the predictive assessment of each ML algorithm on overall survival (OS) as determined by area under the curve (AUC). Pretransplant variables used in the EBMT ML study (Shouval et al., 2015) were used as a benchmark for comparison.</p><p><strong>Results: </strong>On the entire dataset, the random forest (RF) algorithm performed best (AUC 0.71 ± 0.04) compared to the second-best model, logistic regression (LR) (AUC = 0.69 ± 0.04) (p < 0.001). Both algorithms demonstrated improved AUC scores using all 45 variables compared to the limited variables examined by the EBMT study. Survival at 100 days post-HCT using RF on the full dataset discriminated patients into different prognostic groups with different 2-year OS (p < 0.0001). We then examined the ML methods that allow for significant individual variable identification, including LR and RF, and identified matched related donors (HR = 0.49, p < 0.0001), increasing TBI dose (HR = 1.60, p = 0.006), increasing recipient age (HR = 1.92, p < 0.0001), higher baseline Hb (HR = 0.59, p = 0.0002), and increased baseline FEV1 (HR = 0.73, p = 0.02), among others.</p><p><strong>Conclusion: </strong>The application of multiple ML techniques on single-center allogeneic HCT databases warrants further investigation and may provide a useful tool to identify variables with prognostic potential.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"280-291"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41096109","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-11-30DOI: 10.1159/000535500
Justin D Woods, Heidi D Klepin
{"title":"Geriatric Assessment in Acute Myeloid Leukemia.","authors":"Justin D Woods, Heidi D Klepin","doi":"10.1159/000535500","DOIUrl":"10.1159/000535500","url":null,"abstract":"<p><strong>Background: </strong>Acute myeloid leukemia (AML) is a heterogenous disease that affects mostly older adults with varying baseline health and functional status. Treatment options have expanded for older adults, ranging from less intensive chronic therapies to intensive induction strategies with curative intent. Despite this, outcomes remain poor with advancing age due to underlying disease biology and variability in treatment tolerance. Reliance on chronological age alone, however, increases risks of both over- and under-treatment. Strategies to better characterize fitness in the context of therapy are needed to optimize decision-making and enhance clinical trial design.</p><p><strong>Summary: </strong>Geriatric assessment (GA) is a series of validated tools that evaluate multiple health and functional domains of an older adult including physical function, comorbidities, cognition, nutrition, psychological health, and social support. While studies of GA in AML remain limited, current evidence shows that it is feasible to perform GA among older adults starting therapy for AML. GA measures including those assessing physical function, cognition, and mood are associated with mortality and toxicity in both intensive and less intensive treatment settings.</p><p><strong>Key messages: </strong>In this review, we discuss the existing evidence to support use of GA in AML and highlight implications for clinical practice and future research.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"219-228"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10963150/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138457140","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-11-30DOI: 10.1159/000535463
Amanda L Blackmon, Christopher S Hourigan
{"title":"Test Then Erase? Current Status and Future Opportunities for Measurable Residual Disease Testing in Acute Myeloid Leukemia.","authors":"Amanda L Blackmon, Christopher S Hourigan","doi":"10.1159/000535463","DOIUrl":"10.1159/000535463","url":null,"abstract":"<p><strong>Background: </strong>Measurable residual disease (MRD) test positivity during and after treatment in patients with acute myeloid leukemia (AML) has been associated with higher rates of relapse and worse overall survival. Current approaches for MRD testing are not standardized leading to inconsistent results and poor prognostication of disease. Pertinent studies evaluating AML MRD testing at specific times points, with various therapeutics and testing methods are presented.</p><p><strong>Summary: </strong>AML is a set of diseases with different molecular and cytogenetic characteristics and is often polyclonal with evolution over time. This genetic diversity poses a great challenge for a single AML MRD testing approach. The current ELN 2021 MRD guidelines recommend MRD testing by quantitative polymerase chain reaction in those with a validated molecular target or multiparameter flow cytometry (MFC) in all other cases. The benefit of MFC is the ability to use this method across disease subsets, at the relative expense of suboptimal sensitivity and specificity. AML MRD detection may be improved with molecular methods. Genetic characterization at AML diagnosis and relapse is now standard of care for appropriate therapeutic assignment, and future initiatives will provide the evidence to support testing in remission to direct clinical interventions.</p><p><strong>Key messages: </strong>The treatment options for patients with AML have expanded for specific molecular subsets such as FLT3 and IDH1/2 mutated AML, with development of novel agents for NPM1 mutated or KMT2A rearranged AML ongoing, but also due to effective venetoclax-combinations. Evidence regarding highly sensitive molecular MRD detection methods for specific molecular subgroups, in the context of these new treatment approaches, will likely shape the future of AML care.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"133-146"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10963159/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138457141","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2023-11-30DOI: 10.1159/000535574
Shai Shimony, Richard M Stone
{"title":"Untangling Complexities of Acute Myeloid Leukemia Review Series.","authors":"Shai Shimony, Richard M Stone","doi":"10.1159/000535574","DOIUrl":"10.1159/000535574","url":null,"abstract":"","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"119-121"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138457142","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}
Acta HaematologicaPub Date : 2024-01-01Epub Date: 2024-02-15DOI: 10.1159/000536650
Michael Iarossi, Marie-Christiane Madeleine Vekemans, Nicolas Weynants, Cedric Hermans
{"title":"Acquired von Willebrand Syndrome Associated with a Smoldering Multiple Myeloma, Successfully Treated by Daratumumab, Lenalidomide, and Dexamethasone.","authors":"Michael Iarossi, Marie-Christiane Madeleine Vekemans, Nicolas Weynants, Cedric Hermans","doi":"10.1159/000536650","DOIUrl":"10.1159/000536650","url":null,"abstract":"<p><strong>Introduction: </strong>Acquired von Willebrand syndrome (AvWS) is a rare entity with approximately 700 cases described in the literature. A number of etiologies are responsible for this condition, mainly lymphoproliferative, myeloproliferative syndromes and cardiac diseases. Management is aimed at preventing and treating bleeds, as well as treating the underlying pathology. In the case of a monoclonal gammopathy, there are limited evidence and high heterogeneity only based on old case reports, resulting in poor quality recommendations. It seems essential in 2023 to take into account and offer the new anti-myeloma treatments available.</p><p><strong>Case presentation: </strong>We describe the case of a patient with an AvWS secondary to an IgG smoldering multiple myeloma, experiencing multiple bleeding, treated successfully with daratumumab, lenalidomide, and dexamethasone, after multiple treatment failure.</p><p><strong>Conclusion: </strong>Daratumumab, lenalidomide, and dexamethasone was demonstrated as a rapid and effective treatment for a patient with severe AvWS and multiple bleeding complications.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"587-591"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139740143","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}