American Journal of Hematology最新文献

{"title":"Hereditary Spherocytosis due to an SPTA1 Nonsense Mutation Coinherited With α spectrinLELY in Trans","authors":"María‐Angustias Molina‐Arrebola, Barbara J. Bain","doi":"10.1002/ajh.70021","DOIUrl":"https://doi.org/10.1002/ajh.70021","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"35 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144715587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous Extramedullary Hematopoiesis in a Patient With Myelofibrosis","authors":"Marko Lucijanic, Luka Manojlovic, Cedna Tomasovic‐Loncaric","doi":"10.1002/ajh.70024","DOIUrl":"https://doi.org/10.1002/ajh.70024","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"22 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144715609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Practical Guidance on Clinical Management of Belantamab Mafodotin-Associated Ocular Events","authors":"Evangelos Terpos, Suzanne Trudel, María-Victoria Mateos, Nicolás Alejandre, Kathryn Colby, Meletios A. Dimopoulos, Simona Degli Esposti, Asim V. Farooq, Maria Gavriatopoulou, Francesca Gay, Vania Hungria, K. Martin Kortüm, Xavier Leleu, Sagar Lonial, Hang Quach, Lugui Qiu, Karthik Ramasamy, Kazuhito Suzuki, Katja C. Weisel, Paul Richardson","doi":"10.1002/ajh.70015","DOIUrl":"10.1002/ajh.70015","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Importance</h3>\u0000 \u0000 <p>Belantamab mafodotin (belamaf)-containing regimens are effective treatment options for patients with relapsed and/or refractory multiple myeloma. Ocular events are a common adverse effect of belamaf treatment, which can be managed by physicians, following appropriate training, to minimize their impact on the patient.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To develop clinical recommendations for the identification, monitoring, and management of ocular events associated with belamaf therapy, and guidance for any required dose modification.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Evidence Review</h3>\u0000 \u0000 <p>A systematic literature review was conducted and an international group of hematologists and ophthalmologists reviewed clinical trial data, real-world evidence, and published literature on belamaf-associated ocular events. This literature review and the collective experience and expertise of the panel of experts informed the recommendations.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Findings</h3>\u0000 \u0000 <p>Belamaf-associated ocular events are common side effects of treatment, which are managed primarily with appropriate dose modification and decreased frequency of dosing, as well as the use of artificial tears. These events affect the corneal epithelium, are mainly Grade 1 and 2 per the Keratopathy and Visual Acuity scale and are reversible in almost all patients. In general, before initiating belamaf therapy, all patients should undergo a baseline ophthalmic evaluation with an eye specialist. However, if there are delays in obtaining ophthalmic evaluation, or if a patient is rapidly progressing, treatment should be initiated, and ophthalmic evaluation should be undertaken as soon as possible. Patients should be also evaluated by an eye specialist before administering the next three belamaf doses (i.e., before Cycles 2, 3, and 4); dose modifications, as described in this paper, may apply if required. Importantly, modifying the belamaf dose in response to an ocular event is not associated with any reductions in treatment efficacy. After Cycle 4, the treating physician may use the Vision-Related Anamnestic tool, alongside clinical judgment, to decide whether to administer the next dose of belamaf or to refer the patient to an eye specialist (i.e., if the patient experiences new worsening of vision or if the ocular events have neither improved after 8 weeks nor resolved after 12 weeks).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions and Relevance</h3>\u0000 \u0000 ","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1839-1850"},"PeriodicalIF":9.9,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.70015","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144715589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Clinically Applicable Model Using Blood Counts to Support the Diagnosis of Prefibrotic Myelofibrosis Versus Essential Thrombocythemia","authors":"Tiziano Barbui,&nbsp;Jürgen Thiele,&nbsp;Arianna Ghirardi,&nbsp;Hans Michael Kvasnicka,&nbsp;Umberto Gianelli,&nbsp;Daniel A. Arber,&nbsp;Heinz Gisslinger,&nbsp;Alessandro M. Vannucchi,&nbsp;Attilio Orazi,&nbsp;Ayalew Tefferi","doi":"10.1002/ajh.70017","DOIUrl":"10.1002/ajh.70017","url":null,"abstract":"<div>\u0000 \u0000 <p>This study aimed to distinguish between essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (pre-PMF) using routine blood tests, with a focus on white blood cell (WBC) and platelet (PLT) levels. We evaluated the predicted probability of a pre-PMF diagnosis based on the interaction between WBC and PLT levels using data from a logistic regression model involving 891 patients with ET and 180 patients with pre-PMF. Patients were divided into four groups based on whether their WBC and PLT values were below or above the respective thresholds of 8.85 × 10⁹/L and 793 × 10⁹/L. The results showed that patients with low levels of both WBCs and PLTs had the lowest predicted probability of pre-PMF (6%), indicating a profile more typical of ET. When either WBC or PLT levels were elevated, the probability increased to approximately 18%–19%, indicating a potential shift toward pre-PMF features. Notably, patients with elevated levels of both WBCs and PLTs had the highest probability of a pre-PMF diagnosis (26%), which was more than four times higher than that of the low–low group. The association between combined WBC/PLT levels and a pre-PMF diagnosis remained significant (<i>p</i> &lt; 0.001), even when adjusting for serum lactate dehydrogenase (LDH) and splenomegaly. These findings suggest that elevated WBC and PLT levels together can serve as a practical and accessible diagnostic tool for supporting the differential diagnosis of ET or pre-PMF in cases of uncertainty, and for deferring bone marrow biopsy in cases of early disease presenting with isolated thrombocytosis.</p>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1772-1778"},"PeriodicalIF":9.9,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144710636","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pseudo-Chédiak-Higashi Inclusions in an Anaplastic Large Cell Lymphoma.","authors":"Radu Chiriac,Lucile Baseggio","doi":"10.1002/ajh.70016","DOIUrl":"https://doi.org/10.1002/ajh.70016","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"214 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroleukemiosis Masquerading as Drug Toxicity in an Adolescent With Refractory AML","authors":"Nia Choi,&nbsp;Deborah Schady,&nbsp;Tim Lotze,&nbsp;Jill Ann Jarrell,&nbsp;Alexandra Rodriguez-Hernandez,&nbsp;Sandra Aziz,&nbsp;Karen K. Moeller,&nbsp;Joanna S. Yi,&nbsp;Suzanne Woodbury,&nbsp;Andrea N. Marcogliese,&nbsp;Zoann E. Dreyer,&nbsp;Eric S. Schafer","doi":"10.1002/ajh.70006","DOIUrl":"10.1002/ajh.70006","url":null,"abstract":"&lt;p&gt;\u0000 &lt;b&gt;A 15-year-old male presented with the acute onset of fever, fatigue, headaches, hyperleukocytosis, anemia, and thrombocytopenia and was ultimately diagnosed with a t(9;11) [KMT2A::MLLT3] KMT2A-rearranged (KMT2A-r) acute myeloid leukemia (AML). Head computed tomography (CT) showed a 4 mm intracranial lesion presumed to represent hemorrhage, and diagnostic lumbar puncture (LP) noted no evidence of leukemia in the cerebrospinal fluid (CSF). The patient started therapy, during which he achieved a minimal residual disease (MRD) negative complete remission at the end of Induction I&lt;/b&gt;.&lt;/p&gt;&lt;p&gt;The patient and family were offered therapy on a Children's Oncology Group clinical trial (AAML1831, NCT04293562), but they chose to be treated on the local best practice standard, which was based largely on the standard arm of that study. The patient's &lt;i&gt;KMT2A::MLLT3&lt;/i&gt; fusion and post-Induction 1 (Cycle 1) MRD negative response placed him into a “Low Risk 2” category for which he would subsequently receive four additional 28-day cycles of chemotherapy termed Induction 2, Intensification 1, Intensification 2, and Intensification 3 without hematopoietic stem cell transplant providing an estimated 5-year disease free survival of 63.7% ± 4.5% [&lt;span&gt;1&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;\u0000 &lt;b&gt;After recovery from Induction I, the patient received Induction II therapy with minimal complications; however, before Intensification I, the patient presented with new left-sided facial weakness. Brain magnetic resonance imaging (MRI) showed multifocal lesions throughout the supratentorial white matter. A biopsy of the area revealed myeloid sarcoma. High-dose cytarabine (HD-AraC) with asparaginase (“Capizzi AraC”) was initiated for relapsed disease, during which extremity pain and global weakness developed, suggestive of a multifocal mixed motor and sensory neuropathy. The patient was started on acetaminophen, morphine, and gabapentin with steady improvement in pain. Physical and occupational therapy (PT and OT) enabled the patient to comfortably ambulate laps with a platform walker. Post-therapy, bone marrow (BM) MRD and CSF were both negative for leukemia, and a brain MRI showed interval improvements of intracranial lesions&lt;/b&gt;.&lt;/p&gt;&lt;p&gt;The patient experienced a surprising, early, on-therapy, isolated central nervous system (CNS) relapse of his AML. Post-infusion asparaginase potentiates the antileukemic effect of HD-AraC and is effective in treating CNS disease [&lt;span&gt;2&lt;/span&gt;]; and so, this “Capizzi II” regimen is commonly used in both de novo (often as part of Intensification therapy) [&lt;span&gt;3&lt;/span&gt;] and relapsed AML [&lt;span&gt;2&lt;/span&gt;] regimens. However, CNS toxicities are well described and include headache, seizure, and somnolence [&lt;span&gt;4&lt;/span&gt;]. Cytarabine-induced peripheral neuropathies have also been reported [&lt;span&gt;4-6&lt;/span&gt;]. While the etiology of his neuropathies was unconfirmed, it was presumed to be drug exposure [&lt;span&gt;7&lt;/span&gt;] versus chronic illness","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1853-1858"},"PeriodicalIF":9.9,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.70006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144684353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Case for Synthetic Images Generated by Artificial Intelligence","authors":"Bingwen Eugene Fan,&nbsp;Stefan Winkler","doi":"10.1002/ajh.70019","DOIUrl":"10.1002/ajh.70019","url":null,"abstract":"&lt;p&gt;The latest deep learning models boast powerful capabilities for data generation in various modalities, including most notably text and images. A recent editorial by Bucci and Parini [&lt;span&gt;1&lt;/span&gt;] focused on the opportunities this creates for researchers acting in bad faith, using such tools for image falsification and scientific fraud. As authors cited in this context [&lt;span&gt;2&lt;/span&gt;], we offer a necessary counterpoint guided by Melvin Kranzberg's first law: “Technology is neither good nor bad; nor is it neutral” [&lt;span&gt;3&lt;/span&gt;]. There is no doubt that this new technology can lead to ethical challenges for users. The downsides, such as the easy production of “deep fake” images and videos, is clearly a worrying trend, posing ethical challenges. However, we must not forget the beneficial use cases of these generative tools with transformative scientific applications already advancing our field.&lt;/p&gt;&lt;p&gt;The development of powerful diffusion models—which reverse information loss through noise addition to generate images that are very similar in distribution to the original dataset they were trained with—represents a technical breakthrough in synthetic image generation. This approach can be considered part of a set of more general techniques, collectively termed &lt;i&gt;data augmentation&lt;/i&gt;, used to enhance training datasets through various transformations in order to increase the quantity and diversity of training images. Basic transformations include geometric distortions, color adjustment, noise injection, filtering, and others. More advanced methods based on deep neural networks have also been developed, such as style transfer, super-resolution, or in-painting [&lt;span&gt;4&lt;/span&gt;]. Synthetic image generation is a natural next step in this process. When ethically deployed, augmentation and generation methods can significantly improve machine learning performance, robustness, and generalization.&lt;/p&gt;&lt;p&gt;This is particularly vital in hematology, where three critical constraints converge: scarce protected patient data, limited rare-disease cohorts, and costly expert annotations. Here, synthetic images offer demonstrable solutions. Firstly, synthetic images of cells from bone marrow smears [&lt;span&gt;5&lt;/span&gt;] and peripheral blood films [&lt;span&gt;2, 6&lt;/span&gt;] enable cross-institutional collaboration without breaching patient confidentiality. Secondly, combining synthetic and real microscopic cell images enhances classification accuracy in diagnostics [&lt;span&gt;7&lt;/span&gt;]. Lastly, augmented datasets reduce reliance on scarce annotated samples while improving model generalizability [&lt;span&gt;4&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;We agree that malevolent use demands governance—clear labeling, provenance documentation, and algorithmic transparency are essential. Generative models remain imperfect; ensuring synthetic images are realistic, diverse, and non-inferential requires ongoing refinement across modalities [&lt;span&gt;8&lt;/span&gt;]. Yet safeguards are advancing: the Nature portfolio of journals stat","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1910-1911"},"PeriodicalIF":9.9,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.70019","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144684355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Perfect Storm for Bleeding: Concurrent Deficiencies of Vitamin C, Selenium, and Zinc in a Young Adult","authors":"Ranjini Vengilote,&nbsp;Zeni Kharel,&nbsp;Peter Kouides","doi":"10.1002/ajh.70020","DOIUrl":"10.1002/ajh.70020","url":null,"abstract":"&lt;p&gt;We describe, to our knowledge, the first reported case of concurrent vitamin C, selenium, and zinc deficiencies manifesting as spontaneous calf hematoma and profound anemia in a young adult with extreme dietary selectivity. This case underscores the importance of considering micronutrient deficiencies in the differential diagnosis of unexplained bleeding and anemia, particularly in patients with restrictive eating patterns and unremarkable coagulation studies.&lt;/p&gt;&lt;p&gt;A 21-year-old male with a history of anxiety on stable fluoxetine presented with 1 week of spontaneous left calf bruising, swelling, and progressive leg pain causing difficulty with ambulation. This was associated with gingival bleeding for the same duration. He also reported a year-long history of petechiae on his arms and legs. He denied any history of trauma, gastrointestinal bleeding, or a personal or family history of bleeding disorders. Over the past year, his diet had been highly restrictive, limited to one meal per day and occasional snacks of crackers and pretzels, with a complete exclusion of leafy greens, red meat, and minimal intake of fruits. He denied alcohol use or a history of eating disorders but described himself as a picky eater. On presentation, he was hemodynamically stable but underweight, with a body mass index (BMI) of 18.12 kg/m&lt;sup&gt;2&lt;/sup&gt;. Physical examination revealed a swollen left calf with extensive ecchymosis extending above the knee and perifollicular hemorrhages on his limbs (Figure 1).&lt;/p&gt;&lt;p&gt;Laboratory studies demonstrated hemoglobin (Hb) of 7.8 g/dL (reference range [RR]: 13.0–18.0 g/dL), mean corpuscular volume (MCV) of 80 fL (RR: 80–100 fL), with normal white blood cell and platelet counts. Last complete blood count done 5 years ago showed Hb of 15.1 g/dL with MCV of 81.5 fL. Coagulation studies showed a borderline elevated international normalized ratio (INR) of 1.2 (RR: 0.9–1.1) and a normal activated partial thromboplastin time (aPTT). Initial laboratory evaluation showed mild elevations in total bilirubin (3.9 mg/dL; RR: 0.3–1.2 mg/dL) and indirect bilirubin (2.8 mg/dL; RR: 0.1–1 mg/dL), normal albumin level and an elevated reticulocyte count (152 × 10&lt;sup&gt;3&lt;/sup&gt;/μL; RR: 28–139 × 10&lt;sup&gt;3&lt;/sup&gt;/μL). Peripheral blood smear review was unremarkable. Hematologic workup for hemolysis was unremarkable, with a normal disseminated intravascular coagulation (DIC) panel, lactate dehydrogenase (LDH), haptoglobin, and a negative direct Coombs test.&lt;/p&gt;&lt;p&gt;Imaging, including an x-ray of the left knee and Doppler ultrasound, revealed no acute osseous abnormalities or deep vein thrombosis. Computed tomography (CT) angiogram of the left lower extremity demonstrated soft tissue swelling of the posterior compartment musculature of the left leg involving gastrocnemius and soleus muscles concerning for intramuscular hematoma without evidence of active extravasation.&lt;/p&gt;&lt;p&gt;Evaluation for inherited and acquired bleeding disorders was unremarkable except for mild","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1906-1909"},"PeriodicalIF":9.9,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.70020","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144684354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemophagocytic Lymphohistiocytosis in Pregnancy: A Systematic Review of Case Reports and Case Series","authors":"Ali Abdelhay,&nbsp;Basant Eltaher,&nbsp;Mouna Reghis,&nbsp;Aditya Sanjeevi,&nbsp;Aniket-vijay Rao,&nbsp;Zachary Teibel,&nbsp;Daniel Grace,&nbsp;Peter Kouides","doi":"10.1002/ajh.70013","DOIUrl":"10.1002/ajh.70013","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome caused by excessive immune activation, leading to severe inflammation and multi-organ failure. While adult-onset HLH is well documented, pregnancy-related HLH remains under investigated, with limited data on its triggers, presentation, and management. We systematically review cases of pregnancy-related HLH in the literature, focusing on presentations, triggers, utilized treatments, and outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A systematic search of PubMed and Embase identified case reports and series describing HLH during pregnancy or postpartum. Data were extracted and analyzed using standardized methods, including frequency distributions for categorical variables and means with standard deviations for continuous variables. Quality was assessed using the Joanna Briggs Institute checklist. Statistical analysis was conducted using IBM SPSS version 26.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The initial search identified 838 records, from which 66 case reports and 7 case series were included, covering 104 unique patients. The mean age of the patients was 29 years (SD 5.6 years). Presentations occurred between 7 weeks of gestation and up to 7 months after delivery; of these, 82.5% presented antepartum at an average gestational age of 24 weeks (SD 7.9 weeks), while 17.5% presented postpartum. Fever (100%), anemia (90.7%), and elevated ferritin levels (&gt; 1000 ng/mL in 95.1%) were the most common findings. Infections were the most frequent triggers (42 patients, 40.4%), with Epstein–Barr virus (EBV) being the most frequently implicated pathogen (10 patients, 9.6%). Other triggers included systemic lupus erythematosus (10 patients, 9.6%), Adult-onset Still's Disease (7 patients, 6.7%), lymphoma (6 patients, 5.8%), and no identifiable trigger in 42 patients (40.4%). Regarding treatment, corticosteroids were used in 93.3% of cases, etoposide in 35.6%, cyclosporine in 17.3%, and intravenous immunoglobulins (IVIG) in 26%. Delivery stabilized or reversed the disease in 40.4% of cases. However, 45.6% of patients with prepartum onset experienced pregnancy loss. Nineteen patients (18.3%) died due to HLH.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This review highlights the distinct characteristics and challenges of HLH in pregnancy. Early diagnosis, a multidisciplinary approach, and individualized treatment strategies are crucial for improving maternal and fetal outcomes.</p>\u0000 </section>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 10","pages":"1828-1838"},"PeriodicalIF":9.9,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144684665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic Stratification","authors":"Théo Simon, Laurent Savale, Kristoffer Grundtvig Skaarup, Paul Breillat, Luu‐Ly Pham, Seyed‐Mehdi Nouraie, Niklas Dyrby Johansen, Jocelyn Inamo, Francois Lionnet, Gylna Loko, Christelle Chantalat, Anne Laure Pham Hung d’Alexandry d’Orengiani, Anoosha Habibi, Gonzalo de Luna, Sihem Iles, Frédéric Galactéros, Etienne Audureau, Tor Biering‐Sørensen, Pablo Bartolucci, Geneviève Derumeaux, Thomas d’Humières","doi":"10.1002/ajh.27768","DOIUrl":"https://doi.org/10.1002/ajh.27768","url":null,"abstract":"Cardiovascular complications are the leading cause of mortality in sickle cell anemia (SCA) patients. While extensive data have identified diastolic dysfunction (DD) to increase morbidity and mortality, the unique hemodynamic conditions inherent to SCA challenge the current recommendations to assess diastolic function. Thus, there is an urgent need to refine the echocardiographic definition of DD to improve risk stratification and therapeutic strategies in SCA patients. We analyzed data from the French multicentric Etendard cohort and compared them with an age‐ and sex‐matched control group from the Copenhagen City Heart Study (CCHS). We focused on left ventricular diastolic parameters, specifically lateral e′ velocity (e′ lat), E/e′ ratio, and indexed left atrial volume (LAVi), assessing their association with clinical outcomes over a 12‐year follow‐up. Etendard SCA patients (<jats:italic>n</jats:italic> = 379) had an early impaired diastolic function compared to the CCHS controls (<jats:italic>n</jats:italic> = 672). This was particularly obvious in young SCA patients (<jats:italic>n</jats:italic> = 252, age ≤ 38 years) in whom e′ lat was associated with prognosis (<jats:italic>p</jats:italic> = 0.01), with an optimal cut‐off value below 11 cm/s. Indeed, young SCA patients with DD had a fourfold increased 12‐year mortality rate as compared with SCA patients without DD (16 vs. 4%, <jats:italic>p</jats:italic> &lt; 0.001). Additionally, e′ lat correlated with 6‐min walk test, NT pro‐BNP levels, diastolic blood pressure, and lactate dehydrogenase levels. In young SCA patients, our data contribute to refine the diagnosis of diastolic dysfunction evaluation. We highlight the prognostic value below 11 cm/s of lateral e′ velocity and its association with key contributors of cardiac impairment such as hemolysis and systemic vasculopathy.Trial Registration: <jats:ext-link xmlns:xlink=\"http://www.w3.org/1999/xlink\" xlink:href=\"https://ClinicalTrials.gov\">ClinicalTrials.gov</jats:ext-link> identifier: NCT00434902","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"20 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144677536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}