American Journal of Hematology最新文献

{"title":"Impact of Switching From Race-Based to Race-Neutral Spirometry Reference Equations in Children With Sickle Cell Anemia.","authors":"Jose A Mejias-Figueroa,Mark Rodeghier,Michael R DeBaun","doi":"10.1002/ajh.27704","DOIUrl":"https://doi.org/10.1002/ajh.27704","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"3 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143932772","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":"Hypoferremic Response to Chronic Inflammation Is Controlled via the Hemojuvelin/Hepcidin/Ferroportin Axis and Does Not Involve Hepcidin‐Independent Regulation of FpnmRNA","authors":"Siqi Liu, Sofiya Tsyplenkova, Carine Fillebeen, Kostas Pantopoulos","doi":"10.1002/ajh.27710","DOIUrl":"https://doi.org/10.1002/ajh.27710","url":null,"abstract":"The iron regulatory hormone hepcidin contributes to the pathogenesis of anemia of inflammation (AI) by inhibiting the iron exporter ferroportin in target cells, causing hypoferremia. Under acute inflammation, hepcidin induction requires hemojuvelin (Hjv), a bone morphogenetic protein co‐receptor, while <jats:italic>Fpn</jats:italic> mRNA is also suppressed in a hepcidin‐independent manner. However, it is unclear whether, during chronic inflammation, Hjv and hepcidin‐independent <jats:italic>Fpn</jats:italic> mRNA regulation are critical for hypoferremia and AI. To address these questions, wild type and <jats:italic>Hjv</jats:italic><jats:sup><jats:italic>−/−</jats:italic></jats:sup> mice, a model of hemochromatosis, were fed for 8 weeks an adenine‐rich diet to develop chronic kidney disease (CKD). Renal inflammation, accessed by increased <jats:italic>Il6</jats:italic> mRNA expression, did not differ among genotypes. Hjv disruption did not mitigate the severity of kidney injury but suppressed the inflammatory induction of liver hepcidin. CKD triggered hypoferremia and mild anemia in wild type mice; however, <jats:italic>Hjv</jats:italic><jats:sup><jats:italic>−/−</jats:italic></jats:sup> littermates maintained high serum iron and normal hemoglobin, consistent with a protective effect of Hjv/hepcidin deficiency. Notably, tissue <jats:italic>Fpn</jats:italic> mRNA levels were not affected by the inflammatory milieu of CKD. Following injection of wild type or <jats:italic>Hjv</jats:italic><jats:sup><jats:italic>−/−</jats:italic></jats:sup> mice with heat‐killed <jats:styled-content style=\"fixed-case\"><jats:italic>Brucella abortus</jats:italic></jats:styled-content>, <jats:italic>Fpn</jats:italic> mRNA was suppressed during the acute phase of inflammation but quickly recovered and persisted in the chronic phase. We conclude that Hjv deficiency reduces hepcidin levels and mitigates anemia in the CKD model, providing further support for pharmacological targeting of Hjv for the treatment of AI. Moreover, our data demonstrate that <jats:italic>Fpn</jats:italic> mRNA suppression only occurs under acute but not chronic inflammatory conditions and therefore cannot substantially contribute to AI pathogenesis.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"94 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143930620","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":"The Impact of Allogeneic Hematopoietic Stem Cell Transplantation on Sickle Cell Retinopathy and Maculopathy: A Prospective, Observational Study","authors":"Rajani P. Brandsen, Elisabeth Dovern, Bart J. Biemond, Roselie M. H. Diederen, Erfan Nur","doi":"10.1002/ajh.27705","DOIUrl":"https://doi.org/10.1002/ajh.27705","url":null,"abstract":"&lt;p&gt;Sickle cell retinopathy (SCR) represents a significant ocular manifestation of sickle cell disease (SCD) that can dramatically impair visual acuity. SCR can be divided into non-proliferative SCR and proliferative SCR. Non-proliferative SCR involves abnormalities of the peripheral retina such as vascular occlusion without neovascularization, typical scarring (“black sunbursts”) and intraretinal hemorrhages (“salmon patches”). Proliferative SCR represents the more severe form of SCR, characterized by neovascularization. This can lead to vitreous hemorrhage or retinal detachment, which can temporarily or permanently impair visual acuity. In more recent years, modern imaging techniques such as spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA) have also revealed subclinical changes in the central part of the retina (the macula) in asymptomatic patients with SCD [&lt;span&gt;1&lt;/span&gt;]. These changes are referred to as sickle cell maculopathy (SCM) and include macular thinning, enlargement of the foveal avascular zone (FAZ) and lower vessel densities. Both SCR and SCM are progressive in nature and associated with increasing age [&lt;span&gt;2&lt;/span&gt;].&lt;/p&gt;\u0000&lt;p&gt;Allogeneic hematopoietic stem cell transplantation (HSCT) is the only established curative treatment option for patients with SCD. Improvements in non-myeloablative regimens have led to excellent disease-free and overall survival in transplanted adults with SCD, also in the setting of haploidentical HSCT [&lt;span&gt;3&lt;/span&gt;]. SCD-related organ damage is one of the main indications for HSCT, but data on long-term outcomes regarding the progression of organ complications is scarce [&lt;span&gt;4&lt;/span&gt;]. This is particularly true for adult patients, who frequently already have developed (sub-)clinical organ damage before HSCT.&lt;/p&gt;\u0000&lt;p&gt;Evidence concerning the behavior of SCR and SCM after HSCT is especially scarce. The only study on SCR after HSCT was conducted in children with SCD and did not include SCM [&lt;span&gt;5&lt;/span&gt;]. This leaves a significant gap in understanding how these conditions evolve in adults with SCD undergoing HSCT. We hypothesized that HSCT has the potential to stabilize pre-existing SCR and will prevent the development of new SCR in patients with SCD. Therefore, the aim of this study was to evaluate the natural course of SCR and SCM in an adult cohort of SCD patients undergoing HSCT.&lt;/p&gt;\u0000&lt;p&gt;In this prospective observational study in adults with SCD (HbSS, HbSC, HbSβ&lt;sup&gt;0&lt;/sup&gt; and HbSβ&lt;sup&gt;+&lt;/sup&gt; genotype), retinopathy and maculopathy were assessed before and at least 1 year post-transplant. All SCD patients undergoing non-myeloablative matched sibling donor (MSD) and haploidentical bone marrow transplantation were included [&lt;span&gt;3, 6&lt;/span&gt;]. Patients undergoing MSD transplantation received a 3-month preconditioning with azathioprine/hydroxyurea, followed by alemtuzumab (1 mg/kg) and 3 Gy total body irradiation (TBI) [&lt;span&gt;6&lt;/span&gt;]. The condit","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"27 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143926603","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":"Allogeneic Hematopoietic Stem Cell Transplantation for Elderly Acute Lymphoblastic Leukemia Patients: A Registry Study From the Société Francophone de Greffe de Moelle et Thérapie Cellulaire (SFGM-TC)","authors":"Yves Chalandon,&nbsp;Raynier Devillier,&nbsp;Ariane Boumendil,&nbsp;Stephanie Nguyen,&nbsp;Claude-Eric Bulabois,&nbsp;Patrice Ceballos,&nbsp;Eolia Brissot,&nbsp;Marie-Thérèse Rubio,&nbsp;Hélène Labussière-Wallet,&nbsp;Johan Maertens,&nbsp;Patrice Chevallier,&nbsp;Natacha Maillard,&nbsp;Xavier Poiré,&nbsp;Cristina Castilla-Llorente,&nbsp;Yves Beguin,&nbsp;Jérôme Cornillon,&nbsp;Sébastien Maury,&nbsp;Tony Marchand,&nbsp;Etienne Daguindau,&nbsp;Jacques-Olivier Bay,&nbsp;Pascal Turlure,&nbsp;Magalie Joris,&nbsp;Anne-Lise Menard,&nbsp;Karin Bilger,&nbsp;Gaelle Guillerm,&nbsp;Sylvie François,&nbsp;Ali Bazarbachi,&nbsp;Sylvain Chantepie,&nbsp;Philippe Lewalle,&nbsp;Ambroise Marçais,&nbsp;Michael Loschi,&nbsp;Malek Benakli,&nbsp;Paul Chauvet,&nbsp;Edouard Forcade,&nbsp;Anne Huynh,&nbsp;Marie Robin,&nbsp;Stavroula Masouridi-Levrat","doi":"10.1002/ajh.27701","DOIUrl":"10.1002/ajh.27701","url":null,"abstract":"<div>\u0000 \u0000 <p>There are very limited data regarding the outcomes of elderly patients with acute lymphoblastic leukemia (ALL) who undergo allogeneic hematopoietic stem cell transplantation (alloHSCT). A total of 316 ALL patients aged ≥ 60 years who underwent alloHSCT between 2010 to 2022 were identified in the SFGM-TC registry. The primary objective was to evaluate progression-free survival (PFS), non-relapse mortality (NRM), relapse incidence (RI), and graft-versus-host disease (GvHD)-free relapse-free survival (GRFS), as well as their risk factors. The median age was 63.8 years (range 60–75.8), 49.8% of patients had Philadelphia-positive B-ALL (Ph + ALL), and 70.9% were in first complete remission (CR1) at transplantation. The donor was an unrelated donor in 52.1%, a matched related donor (MRD) in 26.3%, and a haplo-identical donor in 17.7%. Reduced-intensity conditioning (RIC) was administered to 64.6% of patients, while total body irradiation (TBI) was used in 35.8%. The 3-year overall survival (OS) was 46% (95% CI 40%–53%). The 3-year PFS, NRM, RI, and GRFS were 41% (95% CI 35%–48%), 23% (95% CI 18%–28%), 36% (95% CI 31%–42%), and 30% (95% CI 25%–37%), respectively. Multivariable analyses confirmed poorer OS and PFS in patients with advanced disease, with an HR of 1.79 (95% CI 1.22–2.64), <i>p</i> = 0.0032. Additionally, the ALL subtype significantly impacted outcomes, with an HR of 1.99 (95% CI 1.42–2.79) for non-Ph + ALL. This study suggests that alloHSCT is a viable option for elderly ALL patients, as age itself did not impact outcomes. However, advanced disease and non-Ph + ALL were associated with significantly worse survival.</p>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 7","pages":"1173-1184"},"PeriodicalIF":10.1,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914855","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":"Optimizing Outcomes in Relapsed and Refractory Multiple Myeloma: Personalized Approaches and Adverse Event Management","authors":"Eirini Katodritou, Evangelos Terpos, Suzanne Lentzsch, Shaji Kumar","doi":"10.1002/ajh.27646","DOIUrl":"https://doi.org/10.1002/ajh.27646","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"60 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143905545","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":"PIEZO1 Activation-Mediated Generation of Transgene-Free Long-Term Hematopoietic Stem Cells","authors":"Giorgia Scapin,&nbsp;Jennifer L. Cillis,&nbsp;Marie C. Goulard,&nbsp;Taylor C. Patch,&nbsp;Cintia E. Gomez Limia,&nbsp;Yichen Ding,&nbsp;Wenqiang Du,&nbsp;Priyanka R. Dharampuriya,&nbsp;Elliott J. Hagedorn,&nbsp;Heidi Anderson,&nbsp;Gabriel A. Musso,&nbsp;Caitlyn R. Curley,&nbsp;Emily M. Teets,&nbsp;Calum A. MacRae,&nbsp;Lalit Sehgal,&nbsp;Tzung K. Hsiai,&nbsp;Bradley W. Blaser,&nbsp;Dhvanit I. Shah","doi":"10.1002/ajh.27689","DOIUrl":"10.1002/ajh.27689","url":null,"abstract":"<div>\u0000 \u0000 <p>The development of engraftable, long-term reconstituting hematopoietic stem cells (LT-HSC) from human pluripotent stem cells (hPSC) has been a long-sought goal. Since HSCs are formed by a subset of endothelial cells in the ventral part of the dorsal aorta, we analyzed heartbeat-mediated pulsatile displacement experienced by the walls of the dorsal aorta in zebrafish embryos. We found that pulsation-mediated circumferential stretch was restricted to the ventral part of the dorsal aorta and activated Piezo1 to stimulate LT-HSC formation. Stimulation of pulsation or Yoda1-mediated Piezo1 activation promoted the formation of <i>de novo</i> LT-HSCs from hemogenic endothelial cells derived from murine embryos or human pluripotent stem cells. These HSCs gave long-term multilineage reconstitution of hematopoietic cells upon transplantation into immunocompromised mice. The formation of transgene-free human LT-HSCs that can engraft and reconstitute the hematopoietic system will facilitate the generation of off-the-shelf HSCs from hPSCs for use in cellular therapies.</p>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 6","pages":"963-979"},"PeriodicalIF":10.1,"publicationDate":"2025-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143905544","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":"Thrombotic Thrombocytopenic Purpura (TTP) Survivors Exhibit Impaired Stress Perfusion on Cardiac MRI","authors":"Senthil Sukumar,&nbsp;Leah Danish,&nbsp;Yuchi Han,&nbsp;Spero Cataland","doi":"10.1002/ajh.27702","DOIUrl":"10.1002/ajh.27702","url":null,"abstract":"<p>Stress images are on the left and rest images are on the right. The top row is a TTP subject and the bottom row is a control subject. The color maps show myocardial blood flow (MBF) in ml/g/min with brighter colors indicating higher perfusion. Stress imaging of the TTP patient shows decreased MBF compared with control, primarily noted in the subendocardium. The rest images do not show any significant difference in MBF at rest.\u0000 <figure>\u0000 <div><picture>\u0000 <source></source></picture><p></p>\u0000 </div>\u0000 </figure>\u0000 </p>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 7","pages":"1256-1258"},"PeriodicalIF":10.1,"publicationDate":"2025-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27702","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143902935","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":"Thrombocytopenia and Anemia After Cardiac Surgery","authors":"Myriam Beshai, Nour Alhomsi, Theodore E. Warkentin","doi":"10.1002/ajh.27696","DOIUrl":"https://doi.org/10.1002/ajh.27696","url":null,"abstract":"&lt;h2&gt;1 Case Presentation&lt;/h2&gt;\u0000&lt;p&gt;\u0000&lt;b&gt;An 83-year-old Caucasian female with hypertension, dyslipidemia, and diabetes presented to the hospital with acute chest pain, nausea, and diaphoresis; she had a previous history of coronary angioplasty (9 years prior). Acute non-ST segment elevation myocardial infarction (NSTEMI) was diagnosed based upon non-specific T wave abnormalities and high-sensitivity troponin-I rise (peak, 780 ng/mL; reference range [RR], &lt; 40). She received enteric-coated aspirin (160 mg first dose; subsequently, 81 mg daily) and 2.5 mg of fondaparinux daily by subcutaneous injection. Heart catheterization, performed with 3000 units (U) of unfractionated heparin [UFH], revealed triple-vessel coronary artery disease (including 80% stenosis in the left anterior descending artery), with left ventricle function preserved; coronary artery bypass graft (CABG) surgery was scheduled.&lt;/b&gt;\u0000&lt;/p&gt;\u0000&lt;p&gt;CABG surgery is common in the United States (400 000 procedures per year) [&lt;span&gt;1&lt;/span&gt;]. While percutaneous coronary intervention may be used to treat NSTEMI, CABG is often performed for triple-vessel coronary disease with stenosi(e)s greater than 70%. Patlolla and coworkers [&lt;span&gt;2&lt;/span&gt;] reported better long-term survival in patients undergoing CABG surgery within 7 days following NSTEMI versus CABG surgery performed later. This patient was judged to be a relatively good candidate for CABG. This patient was treated in a Canadian hospital, and thus the antithrombin-dependent pentasaccharide anticoagulant, fondaparinux—which is approved in Canada for coronary artery thrombosis prophylaxis in STEMI patients based upon efficacy and safety (versus enoxaparin) in this clinical setting [&lt;span&gt;3&lt;/span&gt;]—was administered (note: fondaparinux is not approved for coronary thrombosis prophylaxis by the U.S. Food and Drug Administration).&lt;/p&gt;\u0000&lt;p&gt;&lt;b&gt;Prior to CABG surgery, the last doses of fondaparinux and aspirin were given 2 and 3 days before surgery, respectively.&lt;/b&gt; &lt;b&gt;Immediately pre-surgery, her complete blood count (CBC) showed: platelet count 200 × 10&lt;sup&gt;9&lt;/sup&gt;/L (reference range [RR], 150–400), hemoglobin 10.8 g/dL (RR, 11.5–16.5), and white blood cell (WBC) count 8.3 × 10&lt;sup&gt;9&lt;/sup&gt;/L (RR, 4.0–11.0), with normal automated WBC differential.&lt;/b&gt; &lt;b&gt;She was typed as blood group A, RhD-positive (A+), without red blood cell (RBC) alloantibodies (by indirect Coombs' test); a direct antiglobulin test (DAT, i.e., direct Coombs) was also negative.&lt;/b&gt; &lt;b&gt;She underwent CABG, with three arteries bypassed, and received a total of 35 000 U of UFH intraoperatively. Her intraoperative and early postoperative course was complicated by bleeding: on the day of surgery, she was transfused a total of 3 units of A+ red cell concentrates (RCCs), and 3 more A+ RCCs during the first two postoperative days.&lt;/b&gt; &lt;b&gt;Her platelet count dropped by 55% to 89 × 10&lt;sup&gt;9&lt;/sup&gt;/L on the day of surgery, and hemoglobin levels dropped by 42% to 6.3 g/dL (nadir) on the day of su","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"34 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143897809","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":"Improved Transplant Outcomes With Alternative Donors in Myelofibrosis: A 20-Year Japanese Registry Analysis of Donor Sources and the Impact of Ruxolitinib","authors":"Kazuki Sakatoku,&nbsp;Hirohisa Nakamae,&nbsp;Makoto Murata,&nbsp;Yutaka Shimazu,&nbsp;Katsuto Takenaka,&nbsp;Fumihiko Kimura,&nbsp;Naoyuki Uchida,&nbsp;Tetsuya Nishida,&nbsp;Takahiro Fukuda,&nbsp;Noriko Doki,&nbsp;Jun Ishikawa,&nbsp;Emiko Sakaida,&nbsp;Keisuke Kataoka,&nbsp;Shuichi Shirane,&nbsp;Masatsugu Tanaka,&nbsp;Hikaru Kobayashi,&nbsp;Masashi Sawa,&nbsp;Toshio Wakayama,&nbsp;Noboru Asada,&nbsp;Yasufumi Uehara,&nbsp;Makoto Yoshimitsu,&nbsp;Junya Kanda,&nbsp;Marie Ohbiki,&nbsp;Yoshiko Atsuta,&nbsp;Takayoshi Tachibana","doi":"10.1002/ajh.27699","DOIUrl":"10.1002/ajh.27699","url":null,"abstract":"<p>This study of 308 myelofibrosis patients shows that in recent years (2013–2019), alternative donors (mismatched unrelated donors and cord blood) achieved survival rates comparable to HLA-matched donors—a significant improvement compared to earlier years (2000–2012) when outcomes differed substantially. Ruxolitinib showed significant benefits in older patients (≥ 57), particularly with mismatched unrelated donors. Cord blood transplantation outcomes improved with MMF-based GVHD prophylaxis. These findings suggest optimized strategies combining both age-specific and donor-specific approaches can maximize transplant success in myelofibrosis patients.\u0000 <figure>\u0000 <div><picture>\u0000 <source></source></picture><p></p>\u0000 </div>\u0000 </figure>\u0000 </p>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 7","pages":"1259-1263"},"PeriodicalIF":10.1,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27699","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143893911","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":"Profound Thrombocytopenia and Dyspnea 11 Days After Cardiac Surgery","authors":"Sebastian Vuong,&nbsp;Menaka Pai,&nbsp;Sarah Patterson,&nbsp;Theodore E. Warkentin","doi":"10.1002/ajh.27691","DOIUrl":"10.1002/ajh.27691","url":null,"abstract":"&lt;p&gt;\u0000 &lt;b&gt;A 59-year-old Caucasian female with hypertension and dyslipidemia presented to the hospital with dyspnea. Her platelet count was 7 × 10&lt;/b&gt;\u0000 &lt;sup&gt;\u0000 &lt;b&gt;9&lt;/b&gt;\u0000 &lt;/sup&gt;\u0000 &lt;b&gt;/L (reference range [RR], 150–400). Eleven days earlier, she had undergone an elective valve-sparing Bentall procedure (replacement of hemi-aortic arch) and ascending aorta repair with cardiopulmonary bypass (CPB) for aortic root aneurysm. Her postoperative course was uneventful, with discharge on postoperative day (POD) 4. The patient reported no bleeding symptoms/signs, and petechiae were not present&lt;/b&gt;.&lt;/p&gt;&lt;p&gt;Platelet count declines are universal post-cardiac surgery, with platelet count recovery to preoperative baseline expected by the seventh POD, and continued platelet count increase that typically peaks by POD14 [&lt;span&gt;1&lt;/span&gt;]; thus, a platelet count of 7 × 10&lt;sup&gt;9&lt;/sup&gt;/L on POD11 is highly abnormal and sufficiently reduced to be classified as “profound” thrombocytopenia (&lt; 20 × 10&lt;sup&gt;9&lt;/sup&gt;/L) [&lt;span&gt;2&lt;/span&gt;]. Yet, this patient presented with dyspnea, not bleeding. The urgent task was to identify promptly the cause of her dyspnea and to address the profound thrombocytopenia with the key question: did a single diagnosis explain both?&lt;/p&gt;&lt;p&gt;\u0000 &lt;b&gt;Vital signs were BP 98/59, HR 102/min, RR 23/min, temperature 38.5°C; oxygen saturation was 95% (room air). Urgent echocardiography showed normal ventricular contractility without valve abnormalities; however, a moderate-to-large pericardial effusion was present; subtle right ventricular diastolic collapse suggested partial or incipient cardiac tamponade. The cardiologists were concerned that the pericardial fluid was blood rather than serous fluid, that is hemorrhagic pericarditis. However, risk of pericardiocentesis was felt to be extremely high due to thrombocytopenia; thus, urgent hematology consultation was requested&lt;/b&gt;.&lt;/p&gt;&lt;p&gt;There is a limited differential diagnosis for profound thrombocytopenia, particularly given the normal platelet count just 11 days earlier. The differential diagnosis includes: pseudothrombocytopenia (spurious thrombocytopenia); consumptive thrombocytopenia (disseminated intravascular coagulation [DIC] secondary to infection/shock, thrombotic microangiopathy [TMA], or immune heparin-induced thrombocytopenia [HIT]); or destructive thrombocytopenia (antibody-mediated platelet clearance by drug-dependent antibodies, autoantibodies, or alloantibodies). A reasonable first diagnostic step is to evaluate the complete blood count (CBC), along with peripheral blood film review.&lt;/p&gt;&lt;p&gt;\u0000 &lt;b&gt;The hemoglobin measured 7.7 g/dL (RR, 13.0–18.0) and the white blood cell (WBC) count was 14.0 × 10&lt;/b&gt;\u0000 &lt;sup&gt;\u0000 &lt;b&gt;9&lt;/b&gt;\u0000 &lt;/sup&gt;\u0000 &lt;b&gt;/L (RR, 4.0–11.0). Repeat CBC confirmed profound thrombocytopenia (8 × 10&lt;/b&gt;\u0000 &lt;sup&gt;\u0000 &lt;b&gt;9&lt;/b&gt;\u0000 &lt;/sup&gt;\u0000 ","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 7","pages":"1234-1239"},"PeriodicalIF":10.1,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ajh.27691","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143893910","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}